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onco + heme
yanivfenig
1207
Medical
Graduate
08/22/2012

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Term
prophylaxis for instent restenosis after bare metal coronary artery stent
Definition
aspirin and plavix for 4 wks
Term
what is the complication of bare metal coronary artery stent within the first month of placement?
Definition
instent stenosis
Term
what % of patients do instent restenosis after bare metal stent despite adequate prophylaxis, what is the cause and alternative Tx?
Definition
25%, plavix resistance, switch to Prasugrel
Term
in restenosis, why is aspirin resistance a bigger problem than plavix resistance?
Definition
it is not helpful to raise doses of add another antiplatelet
Term
what is the erroneous mechanism in VTE vs. arterial thromboembolism?
Definition
coagulation factors vs. platelet activation
Term
a mutation in glycoprotein 1b platelet receptor would increase the risk for which disorder, venous or arterial thrombosis?
Definition
arterial thrombosis which is affected by platelet activation
Term
which patients with hemophilia receive primary prophylaxis?
Definition
severe cases with less than 1% normal factor
Term
in the Tx of bleeding d/t hemophilia what are the desired levels of normal factor?
Definition
mild bleeds: 30-50%
major bleeds: 50%
Term
what is the adjunct Tx for Hemophilia A
Definition
DDAVP
Term
in mastocytosis what % of patients experience skin-limited disease and which experience systemic disease?
Definition
80% skin
20% systemic
Term
what is the MC manifestation of skin mastocytosis and what are 2 other
Definition
MC is cutaneous urticaria pigmentosa
other: diffuse cutaneous mastocytosis and mastocytoma
Term
11 s/s in systemic mastocytosis
Definition
pruritus, dermatographia, rash, abd pain, flushing, HTN, syncope, diarrhea, arthralgia, fractures, eosinophilia
Term
3 major associated diseases in systemic mastocytosis 40%
Definition
CML, myeloproliferative syndrome or MDS
Term
6 signs that suggest poor Px in mastocytosis
Definition
1. increases serum tryptase
2. BM involvement
3. Splenomegaly
4. cyopenia
5. skeletal involvement
6. malabsorption
Term
what is the MC mutation in matocytosis
Definition
C-kit (but gleevec not effective)
Term
what can be said about the epidemiology of the hypereosinophilic syndromes?
Definition
MC in males than females
Term
what 3 things are imperative in the Dx of hypereosinophilic syndrome and chronic eosinophilic leukemia and establishing them is required as the first step before excluding other possible diseases
Definition
1. eosinophilia>1500
2. increased marrow eosinophils
3. myeloblasts (<20%) in blood or marrow for at least 6 months
Term
what is the minimum time to make the Dx of hypereosinophilic syndrome or chronic eosinophilic leukemia?
Definition
6 months (to establish myeloblasts in blood or marrow)
Term
what 4 steps does the Dx of hypereosinophilic syndrome and chronic eosinophilic syndrome consist of?
Definition
1st step- establish imperative facts
2nd step- exclude possible causes of secondary eosinophilia
3rd step- exclude other clonal hypereosinophilic conditions
4th step- distinguish btwn the two
Term
4 conditions of secondary eosinophilia to exclude as the 2nd step in the Dx of hypereosinophilic syndrome and chronic eosinophilic syndrome
Definition
1. allergy
2. parasites
3. pulmonary disease (e.g., hypersensitivity pneumonitis, Loeffler's)
4. collagen vascular diseases
Term
3rd step in the Dx of hypereosinophilic syndrome and chronic eosinophilic syndrome - what should practically be done?
Definition
to exclude other primary malignancies associated with eosinophilia or MPD perform immunoflueroscense and seek mutations in the peripheral blood and cytogenetics for translocations in the BM
Term
4th step in the Dx of hypereosinophilic syndrome and chronic eosinophilic syndrome - how to distinguish btwn them
Definition
check peripheral blood and marrow for blasts:
1. if PB<2% and BM<5% - hypereosinophilic syndrome
2. if PB>2% and BM>5% - chronic eosinophilic leukemia
Term
3 of the MC organ systems to be affected in eosinophil-mediated tissue damage?
Definition
1. heart
2. lungs
3. CNS
Term
after establishing the Dx of a disease associated with eosinophil-mediated tissue damage such as hypereosinophilic syndrome or chronic eosinophilic leukemia, what are the 3 steps in the workup?
Definition
1. echocardiography
2. troponin
3. CXR
Term
which mutated receptor in hypereosinophilic syndrome or chronic eosinophilic leukemia indicates good resopnsiveness to Gleevec
Definition
PDGF
Term
which mutated receptor in hypereosinophilic syndrome or chronic eosinophilic leukemia indicates poor resopnsiveness to chemotherapy?
Definition
FGF
Term
which patients with hypereosinophilic syndrome or chronic eosinophilic syndrome can be only observed?
Definition
asymptomatic patients with the absence of mutation in PDGF
Term
what is the 1st line of Tx for symptomatic hypereosinophilic syndrome and chronic eosinophilic leukemia, and what are 6 alternative Tx options
Definition
1st line is steroids
Hydroxyurea
interferon
cladribine
cyclosporine
anti-IL5 antibody mepolizumab
Anti-CD52 antibody (alemtuzumab)
Term
what serum marker could indicate systemic vs. cutaneous mastocytosis?
Definition
tryptase>20
Term
what is the MC myeloid neoplasm associated with mastocytosis?
Definition
myeloproliferative disorder
Term
what is the median survival of a patient with mastocytosis and an associated myeloid neoplasm?
Definition
2 years
Term
what is the median survival of patients with indolent mastocytosis
Definition
over 16 years
Term
what is the median survival of patients with aggresive mastocytosis w/o associated myeloid neoplasm
Definition
3.5 years
Term
3 line Tx of mastocytosis
Definition
1. Cladiribine
2. Hydroxyurea
3. Interferon alpha
Term
which of the Tx of mastocytosis achieves the longest response?
Definition
hydroxyurea 2.5 yrs
Term
what is the definition of mast cell leukemia?
Definition
over 10% of mast cells in peripheral blood (which occur when mast cells are over 50% in BM)
Term
MCC of mediastinal mass
Definition
thymoma 40%
Term
8 entities on the DD of anterior mediastinal mass
Definition
thymoma, lymphoma, germ cell tumors, substernal thyroid tumors, lipoma, carcinoid, TB, sarcoidosis
Term
4 s/s of thymoma
Definition
respiratory symptoms
chest pain
weight loss/anorexia
night sweats
Term
what % are asymptomatic at presentation of thymoma
Definition
50%
Term
Dx of thymoma
Definition
Bx by mediastinoscopy or limited thoracotomy
Term
why not perform FNA for the Dx of thymoma
Definition
poor in differentiating thymoma from lymphoma
Term
how is the Px of thymoma determined
Definition
by degree of invasion
Term
most thymoma is diagnosed as invasive or non-invasive
Definition
non-invasive
Term
thymoma is associated with what 3 other conoditions MC
Definition
myasthenia gravis, pure red cell aplasia, hypogammaglobulinemia
Term
what Tx is effective in 30% of patients with thymoma and pure red cell aplasia
Definition
thymectomy
Term
in what thymoma associated condition is thymectomy not at all helpful?
Definition
hypogammaglobulinemia
Term
what % of ovarian cancer have BRCA
Definition
10%
Term
what is the risk of developing ovarian cancer with BRCA1
Definition
30-50%
Term
what is the risk of developing ovarian cancer with BRCA2
Definition
20-40%
Term
what is the recommended action if BRCA is found, screening by CA125+US or oopherectomy?
Definition
oopherectomy
Term
by how much does oopherectomy reduce the risk of breast cancer?
Definition
50%
Term
what is the method of screening for Prostate Ca
Definition
annual DRE and PSA
Term
from what age do you screen for prostate ca
Definition
caucasians>50, african-american>45
Term
what is the first step in screening for prostate ca?
Definition
DRE
Term
in screening for prostate ca, what is the next step if DRE is abnormal?
Definition
TRUS guided Bx
Term
in screening for prostate ca, if DRE was abnormal and Trus guided Bx was negative, what is the next step?
Definition
PSA
Term
in screening for prostate ca, if DRE was abnormal, Trus guided Bx was negative, and PSA>4, what is the next step?
Definition
repeat Bx in 3-6 months
Term
in screening for prostate ca, if DRE was normal, what is the next step?
Definition
PSA
Term
in screening for prostate ca, if DRE was normal and total PSA<4 and velocity<0.75, what is the next step?
Definition
annual DRE and PSA
Term
in screening for prostate ca, if DRE was normal and total PSA<4 and velocity>0.75, what is the next step?
Definition
TRUS guided Bx
Term
in screening for prostate ca, if DRE was normal, total PSA<4, velocity>0.75, and Trus guided Bx negative, what is the next step?
Definition
repeat Bx anually
Term
in screening for prostate ca, if DRE was normal and total PSA 4-10, what is the next step?
Definition
optional: 1. Trus guided Bx
2. % Free PSA
Term
in screening for prostate ca, if DRE was normal, total PSA 4-10 and Trus guided Bx was negative, what is the next step?
Definition
%free PSA in 6-12 months
Term
in screening for prostate ca, if DRE was normal, total PSA 4-10 and %free PSA was low, what is the next step?
Definition
Trus guided Bx
Term
in screening for prostate ca, if DRE was normal, total PSA 4-10, %free PSA was low, and Trus guided Bx was negative, what is the next step?
Definition
%free PSA in 6-12 months
Term
in screening for prostate ca, if DRE was normal and total PSA>10, what is the next step?
Definition
Trus guided Bx
Term
in screening for prostate ca, if DRE was normal, total PSA>10 and Trus guided Bx was negative, what is the next step?
Definition
repeat Bx
Term
what is the benefit of screening for prostate ca in survival?
Definition
unclear. 1 in 6 diagnosed, 1-30 dies of the disease
Term
what is the effect on the risk of getting prostate ca if one family member is sick with the disease?
Definition
X2
Term
what is the effect on the risk of getting prostate ca if 2 family members are sick with the disease?
Definition
X4
Term
hereditary prostate ca is correlated with what disease dynamics?
Definition
early onset
Term
what % of prostate ca disease are hereditary
Definition
5-10%
Term
which 2 genes are implicated in prostate ca?
Definition
5 alpha reductase type 2 (SRD5A2)
cytochrome P450 c17
Term
being african american is associated with increases in the incidence of what to things associated with prostate ca? and what is the reason
Definition
larger tumors
prostatic intraepithelial neoplasia (PIN) - precancerous
higher levels of testosterone
Term
4 protective dietary factors for prostate ca
Definition
1. legumes (kitniyot) - 5areductase
2. cruciferous veggies (from the cabage family)
3. tomatoes - retinoids
4. statins - inhibit cholesterol biosynthesis
Term
which food increases the risk of prostate cancer
Definition
red meat
Term
which Tx is the only one considered protective against prostate ca
Definition
5 alpha reductase inhibitors
Term
which 3 studied Tx are considered to have no effect on the risk of developing prostate ca
Definition
vit C, E and selenium
Term
what seemingly has no effect on the PSA, but actually does?
Definition
DRE can increase PSA X10 for 10 wks
Term
how long after prostatectomy will PSA be undetectable
Definition
6 wks
Term
what % of patients with abnormal DRE and a PSA>4 have prostate ca
Definition
25%
Term
what % of patients with normal DRE and a PSA of 2.5-4 have prostate ca
Definition
17%
Term
which is the imaging technique of choice in prostate ca
Definition
MRI
Term
what % of people with prostate cancer a diagnosed with local disease (T1 or T2)?
Definition
80%
Term
what % of patients with prostate cancer are diagnosed with regional disease (T3/T4 with no mets)
Definition
12%
Term
what % of patients with prostate cancer are diagnosed with disseminated disease?
Definition
4%
Term
what is the classic triad in RCC? and how many of the patients present with it?
Definition
hematuria, flank pain and palpable mass
20%
Term
whats more common in RCC anemia or erythrocytosis?
Definition
anemia
Term
how do most cases of RCC present
Definition
incidentally on US/CT
Term
2 conditions ~ with increased risk of RCC
Definition
ESRD
Tuberous Sclerosis
Term
what is the Tx of RCC stage 1/2? and the 5 yr survival?
Definition
radical nephrectomy, 80%
Term
pharma Tx of RCC
Definition
Sutent
Term
what % of TCC develops in the bladder, renal pelvis and ureters/urethra?
Definition
90%, 8%, 2%
Term
3 epidemiologic facts about TCC
Definition
M>F, whites>blacks, median age 65
Term
what is the increase in risk of TCC with smoking? and how long does it persist after quitting?
Definition
X2-4, 10 years
Term
how many of the TCC cases are thought to be related to smoking?
Definition
50%
Term
6 risk factors for TCC
Definition
smoking
phenacetin
aniline dye
schistosoma haematobium
radiation
cyclophosphamide
Term
in TCC, mutations in which 3 genes are associated with increased recurrence, metastasis and death?
Definition
p53, p21 and Rb
Term
in TCC, mutations in which two components is associated with less invasive tumors?
Definition
FGF3 and tyrosine-kinase
Term
in TCC, which mutations in which 2 genes are associated with CIS and invasive disease?
Definition
Rb and p53
Term
what is the MCC of gross hematuria from the bladder? 2nd MCC
Definition
benign cysts, 2nd-TCC of bladder
Term
what is the MC origin of microscopic hematuria?
Definition
prostate (much more than bladder)
Term
3 needed steps in the workup of hematuria
Definition
urinary cytology, imaging of bladder by CT/intravenous pyelogram, cystoscopy
Term
what 5 steps should be taken in cystoscopy for suspected TCC of bladder?
Definition
1. documentation of tumors
2. resection of all tumors
3. Bx from underlying muscle of each tumor resected
4. random Bx from healthy appearing areas
5. if no tumor visible in bladder but cytologies positive, catheterization and visualization of the ureters
Term
2 uses of intravesical therapy in superficial TCC?
Definition
1. prevent recurrence
2. eliminate disease that wasn't completely eliminated by resection
Term
in superficial TCC, what is the rate of recurrence of disease after resection?
Definition
50%
Term
what % of superficial TCC after resection progress to advanced disease?
Definition
up to 20%
Term
4 conditions where intravesical therapy is recommended in superficial TCC
Definition
recurrent disease, >40% involvement of the bladder surface by tumor, diffuse CIS, or T1 disease
Term
4 possible drugs used in intravesical therapy for superficial TCC?
Definition
BCG, IFN, mitomycin-C, Gemcitabine
Term
2 general side effects of intravesical therapy for the Tx of superficial TCC
Definition
dysuria and urinary frequency
Term
how do you monitor the recurrence of superficial TCC post-resection
Definition
a full (urethra to renal pelvises) endoscopic examination every 3 months for one year
Term
what happens when the Tx of superficial TCC is very "successful" in the bladder?
Definition
the disease tends to recur more frequently extravesicularly (ureters and urethra)
Term
2 Tx options of persistent or new tumors of (post resection) superficial TCC
Definition
1. repeat BCG or other intravesical therapy
2. cystectomy
Term
4 CIs to neobladder after cystectomy in invasive TCC
Definition
1. CIS in the urethra
2. inability to self catheterize
3. exophytic tumor
4. renal insufficiency
Term
4 conditions to perform partial cystectomy in invasive TCC
Definition
1. disease limited to thedome of the bladder
2. a margin of 2 cm
3. adequate remaining bladder capacity
4. no CIS in other areas
Term
6 types of possible bladder malignancies
Definition
1. TCC
2. SCC
3. adenocarcinoma
4. small cell
5. melanoma
6. lymphoma
Term
which symptoms in TCC are suggestive for in situ disease?
Definition
irritative symptoms
Term
the recurrence and Px in TCC is dependent on what 3 factors
Definition
pathologic stage, lymph node and metastasis
Term
what are the 3 factors that hinder complete remission in metastatic TCC
Definition
1. karnofsky performance status < 80
2. nodal spread
3. visceral spread
the more factors the less remission
Term
which 3 organs does metastatic TCC involve?
Definition
liver, lung, bone
Term
3 standard chemotherpeutic regimens for the Tx of metastatic TCC
Definition
1. M-VAC - MTX, vinblastine, adriamycin, cisplatin
2. Gemcitabine, paclitaxel, cisplatin
3. Gemcitabine, cisplatin
Term
in the Tx of metastatic TCC which side effects are typical of M-VAC and which of GC
Definition
M-VAC: mucositis, fever, neutropenia
GC: anemia, thrombocytopenia
Term
which is a more efficient Tx of metastatic TCC, GC or GTC?
Definition
both are equally efficient
Term
in TCC what is the effect of adjuvant/neoadjuvant therapy?
Definition
improves 5 yr survival and median survival
Term
2 risk factors for pelvic and ureter TCC
Definition
1. balkan nephropathy - interstitial
2. chronic phenacetic use
Term
how is the Dx of pelvic/ureter TCC usually made?
Definition
during the workup of painless hematuria with intravenous pyelogram
Term
how is low grade TCC of the renal pelvis/ureter treated?
Definition
nephroureterectomy - including a part of the bladder
Term
what is the 5 year survival after Tx of low grade renal pelvis/ureter TCC
Definition
80-90%
Term
what % of patients with VHL develop clear cell renal cell carcinoma?
Definition
35%
Term
besides RCC, what other (6) neoplasms are associated with VHL?
Definition
1. retinal hemangioma
2. hemangioblastoma of the spinal cord and cerebellum
3. pheochromocytoma
4. neuroendocrine tumors and cysts
5. epididymal cysts
6. broad ligament cysts
Term
4 paraneoplastic conditions seen in RCC
Definition
1. dysfibrinogenemia
2. hypercalcemia
3. erythrocytosis
4. non-metastatic hepatic dysfunction
Term
4 steps in the workup of a patient suspected of RCC and a 5th optional step
Definition
1. abdominal CT
2. CXR
3. urine analysis
4. urine cytology
5. if suspected involvement of IVC - MRI
Term
5 malignancies that may involve the kidney
Definition
1. RCC
2. TCC
3. lymphoma
4. wilm's tumor
5. sarcoma
Term
4 stages of RCC according to the American joint committee on cancer
Definition
1. <7 cm confined to kidney
2. >7 cm confined to kidney
3. invades perinephric tissue but confined to Gerotas fascia or invades IVC or regional lymph node involvement
4. invades Gerotas fascia or mets
Term
Tx of localized RCC stage 1-3
Definition
radical nephrectomy which includes everything inside gerotas fascia: kidney, adrenal and lymph nodes and tumor invading IVC
Term
2 situations where partial nephrectomy d/t RCC should be considered
Definition
1. bilateral RCC
2. one kidney left
Term
what is the role of adjuvant Tx in partial nephrectomy d/t RCC?
Definition
none, doesn't change outcome
Term
2 indications for nephrectomy in the RCC metastatic patients
Definition
1. alleviate pain and hemorrhage
2. cytoreduction of disease before systemic Tx
Term
what is the role of chemotherapy in metastatic RCC?
Definition
none, it is refractory
Term
what are 2 components of cytokine therapy in metastatic RCC and what % of patients produce regression?
Definition
1. il2
2. IFN alpha
10-20%
Term
2 1st line Tx in metastatic RCC
Definition
Sunitinib (Sutent)
Sorafenib(Nexavar)
Term
a common side effect for Sunitinib and sorafenib used in the Tx of metastatic RCC and 2 additional side effects of sorafinib
Definition
diarrhea
rash, hand-foot syndrome
Term
2 alternative Tx to metastatic RCC patients refractory to Sorafenib/Sunitinib
Definition
mammalian Target of Rapamycin inhibitors (mTOR inhibitors) Temsirolimus and Everolimus
Term
how do Sunitinib and Sorafenib work?
Definition
inhibit Receptor Tyrosine Kinase signalling in VEGF/PDGF receptors
Term
5 poor prognostic factors in metastatic RCC
Definition
1. no prior nephrectomy
2. Kornofsky performance status < 80
3. hypercalcemia
4. increased LDH
5. low Hb
Term
5 yr survival of all stages combined in pancreatic ca?
Definition
5%
Term
what % of patients with pancreatic ca present with inoperable or metastatic disease?
Definition
90%
Term
MC risk factor for pancreatic ca and 5 other risk factors
Definition
smoking
hereditary chronic illnesses, chronic pancreatitis, DM, familial multi-organ cancer syndromes, direct hereditabillity
Term
the MC oncogene in pancreatic ca and the MC tumor suppressor gene + 2 other TSGs + 5 other oncogenes
Definition
Kras
p16
p53, SMAD4
Survivin, FAK, c-Src, IGF-1R, PALB2
Term
which genetic abberation is associated with poorer survival after resection in pancreatic ca?
Definition
inactivation of the tumor suppressor gene SMAD4
Term
what % of pancreatic ca is inherited? in what 3 mechanism is it inherited? and which is the MC?
Definition
16%
1. predisposition to chronic disease predisposing to pancreatic ca
2. MC - directly inherited predisposition
3. multiple organ malignancy syndromes
Term
6 familial multi-organ cancer syndromes consisting of pancreatic ca
Definition
1. Li-Fraumeni
2. HNPCC
3. FAP
4. BRCA1/BRCA2
5. Peutz-Jegehrs
6. Familial atypical Multiple Mole Melanoma (FAMMM)
Term
which familial multi-organ cancer syndrome is associated with the highest lifetime risk of pancreatic ca? what is the genetic mutation?
Definition
Peutz-Jegehrs syndrome, STK11
Term
4 hereditary chronic illnesses associated with increased risk of pancreatic ca
Definition
1. CF
2. hereditary pancreatitis
3. DM
4. ataxia telangiectasia
Term
4 situation where there is a consensus for performing pancreatic ca screening, what is common to the 4 of them and the screening method
Definition
4 situations that entail a tenfold risk of pancreatic ca:
1. hereditary pancreatitis
2. peutz jeghers
3. over 3 1st degree family members with pancreatic ca
4. FAMMM
Endoscopic Ultrasound
Term
what is the imaging modality of choice in pancreatic ca?
Definition
dual phase contrast enhanced spiral CT
Term
for what goal is MRI superior to CT? and where are those equal
Definition
assessing spread of disease (not tumor resectibility)
Term
in pancreatic ca, what is the advantage of EUS over the rest of the imaging modalities?
Definition
superior ability to provide local staging - lymph node involvement, vascular invasion, identify lesion smaller than 3 cm
Term
in pancreatic ca, when should FDG-PET be seriously considered?
Definition
prior to surgery or major chemoradiation Tx b/c of its superior ability to detect metastasis
Term
what is the method of choice for obtaining ductal brushings in pancreatic ca?
Definition
ERCP
Term
when is tissue Bx indicated in pancreatic ca (2)? and what is the method of choice for obtaining it?
Definition
1. doubt regarding the Dx of pancreatic ca.
2. prior to neoadjuvant Tx
EUS
Term
why is EUS-FNA preferred over percutaneous FNA for tissue Bx in pancreatic ca?
Definition
less peritoneal dissemination
Term
in pancreatic ca, when is percutaneous FNA acceptable?
Definition
only in metastatic or inoperable disease
Term
4 uses of CA19-9 in pancreatic ca
Definition
1. presurgery prognostic factor
2. post surgery indicator of recurrence
3. postsurgery prognostic factor
4. biomarker of response to chemo in metastatic disease
Term
staging of pancreatic ca
Definition
1. limited to pancreas
2. beyond pancreas or regional lymph nodes (T3 or N1)
3. involves celiac axis or SMA (T4)
4. mets
Term
what % of patients with pancreatic ca have microscopic residual disease post surgery
Definition
30% (R1)
Term
what is the median survival and 5 yr survival for patients who underwent R0 resection in pancreatic ca?
Definition
20-24 months, 20%
Term
what is the cutoff of tumor size in pancreatic ca that entails better prognosis?
Definition
3 cm
Term
what type of surgery for pancreatic ca of the uncinate or head of the pancreas
Definition
pylorus-preserving pancreaticoduodenectomy (modified Whipple's procedure)
Term
what type of surgery for pancreatic ca of the body or tail of the pancreas
Definition
distal pancreatectomy, includes splenectomy.
Term
what is the adjuvant Tx for resection in pancreatic ca in europe vs. USA
Definition
1. europe: 5FU or Gemcitabine
2. USA: 5FU based CRT following Gemcitabine
Term
what is the survival difference btwn 5FU and Gemcitabine
Definition
none
Term
which Tx given post complete resection delays recurrence?
Definition
Gemcitabine
Term
how is Gemcitabine superior to 5FU?
Definition
safety profile - less incidence of stomatitis and diarrhea
Term
what is the adjuvant Tx to Gemcitabine in patients with locally advanced non metastatic pancreatic ca who had reached steady disease after 3-6 months of Tx?
Definition
radiation Tx
Term
what % of patients present with metastatic pancreatic ca?
Definition
60%
Term
what is the standard Tx of metastatic pancreatic ca
Definition
Gemcitabine
Term
in metastatic pancreatic ca, what 2 combinations of Gemcitabine show improved survival compared with Gemcitabine alone?
Definition
1. Gemcitabine + Erlotinib (TARCEVA - a reversible tyrosine kinase inhibitor, which acts on the epidermal growth factor receptor (EGFR) 2. Gemcitabine + Capcitabine (GEM-CAP)
Term
which Tx for metastatic pancreatic ca shows improved response rate compared with Gemcitabine monotherapy?
Definition
GEM-CAP
Term
which patients with metastatic pancreatic ca are eligible for chemotherapy and what regimen?
Definition
only good performance status
FOLFIRINOX - 5FU/FA, Irinotecan, Oxaliplatin
Term
what is the incidence ratio of HCC in males vs. females?
Definition
4:1 with cirrhosis
1:1 w/o cirrhosis
Term
what % of people with HCV develop cirrhosis? out of them, what % develop HCC? out of patients with HCV what % develop HCC annually?
Definition
10%, 5%, 0.5%
Term
2 areas in the world with the greatest incidence of HCC?
Definition
china, sub-saharan africa
Term
5 common risk factors for HCC
Definition
1. cirrhosis
2. HBV/HCV
3. Aflatoxin B1
4. NASH
5. ethanol
Term
8 unusual risk factors for HCC (think substance overload conditions)
Definition
1. alpha1 antitrypsin def
2. hereditary tyrosinemia
3. primary billiary cirrhosis
4. hemochromatosis
5. wilson's disease
6. citrullinemia
7. glycogen storage diseases
8. porphyria cutanea tarda
Term
what is the MC presentation of HCC (in USA)?
Definition
abdominal pain
Term
what is the 2nd MC presentation of HCC?
Definition
routine lab work with LFTs
Term
what % of patients have no cirrhosis prior to Dx of HCC?
Definition
20%
Term
what are 3 tumor characteristics of HCC
Definition
1. 3 or more lesions
2. bilobar
3. portal vein invasion
Term
what is the MC sign of physical exam in HCC
Definition
hepatomegaly
Term
what is the cause of thrombocytopenia and leukopenia in HCC?
Definition
portal hypertension
Term
10 paraneoplastic signs in HCC
Definition
1. hypercholesterolemia
2. hypercalcemia
3. hypoglycemia
4. increased thyroxin-binding globulin
5. increased sex hormone binding globulin
6. leukopenia
7. thrombocytopenia
8. dysfibrinogenemia
9. carcinoid syndrome
10. porphyria cutanea tarda
Term
3 popular staging systems for HCC
Definition
CLIP, Okuda and TNM
Term
4 parameters in the Okuda staging system for HCC?
Definition
1. albumin (cutoff 3)
2. extent of tumor in liver (cutoff 50%)
3. ascites
4. bilirubin (cutoff 3)
Term
5 parameters in the CLIP staging system for HCC?
Definition
1. single or multiple tumors
2. extent of liver involved
3. AFP
4. Child-Pugh score
5. portal vein thrombosis
Term
9 laboratory tests indicated in the workup of a patient with recent hepatic decompensation or palpable liver mass suspected of HCC
Definition
CBC (splenomegaly), LFTs, ammonia, CEA, AFP and DCP (PIVKA-2), Ca2+ and Mg2+ hepatitis B, C, and D serology (and quantitative HBV DNA or HCV RNA, if either is positive); neurotensin (specific for fibrolamellar HCC)
Term
what is PIVKA and except for HCC in what iatrogenic setting is it elevated?
Definition
protein induced by vitamin K absence (des gamma carboxy prothrombin)
Term
what is a good screening tool for HCC and what 2 classic vascular abnormalities does it pick up?
Definition
US, neovascularization and thrombosis
Term
what is the best imaging option to determine size, extent and portal vein invasion by HCC?
Definition
triphasic contrast CT of the abdomen, pelvis and chest
Term
what is the finding on CT in portal vein involvement d/t HCC
Definition
obstruction and expansion of the vessel
Term
what is the advantage of using MRI with the new contrast dye Ethiodiol for HCC
Definition
it stains the tumor cells, so that when Bx is performed you can be certain that you got tissue from the tumor itself
Term
which imaging technique is superior in HCC
Definition
MRI, CT and US are equal
Term
what is the preferred method to obtain tissue Dx in HCC and why?
Definition
core Bx (not FNA) b/c required tissue architecture to distinguish HCC from adenocarcinoma
Term
4 causes of bleeding complication during Bx in HCC
Definition
1. ascites
2. coagulation factor def
3. thrombocypenia
4. hypervascularity
Term
what is the significance of finding portal vein involvement on liver Bx in HCC?
Definition
CI for liver transplantation
Term
what is the benefit of screening for HCC in high risk populations?
Definition
nothing
Term
what is the consensual method of screening for HCC in high risk populations and who are these populations?
Definition
6 monthly AFP and CT/US (or)
HBV carriers, HCV cirrhosis, family history of HCC
Term
what is the mortality risk for major hepatectomy in HCC
Definition
5-10%
Term
what is a preoperative procedure for surgical excision in HCC and what is the rationale for it?
Definition
Preoperative portal vein occlusion to cause the tumor to shrink and the normal tissue to hypertrophy - safer surgery
Term
what are 5 preconditions to perform surgical excision in HCC?
Definition
1. no cirrhosis or child's A cirrhosis
2. single tumor
3. no metastases
4. no ascites
5. no variceal bleeding
Term
what are preconditions to perform liver transplantation in HCC?
Definition
1. any class of cirrhosis
2. single lesion smaller than 5 cm or maximum 3 lesions each smaller than 3 cm (Milan Criteria)
3. no gross vascular invasion
Term
4 factors that are CI to surgery in HCC?
Definition
1. gross vascular invasion
2. more than 3 lesions or a lesion over 6 cm
3. lymph node involvement
4. metastases
Term
in patients with HCC who are candidates for transplantation, how do you determine if for cadaver or live doner?
Definition
UNOS - United Network for Organ Sharing protocol
Term
what is a bridging Tx for patients waiting on the UNOS transplant list (2)?
Definition
1. neoadjuvant
2. Radio Frequency Ablation (RFA) or Transarterial Chemoembolization (TACE) or 90Yttrium - beta emitter
Term
in HCC patients who are not candidates for any surgical intervention, who are the patients eligible for TACE or 90Yttrium (2)?
Definition
1. child's A/B
2. no metastases
Term
what is the Tx of stage 1 or 2 HCC patients with either child B or C cirrhosis, variceal bleeding or ascites?
Definition
liver transplantation
Term
what are the 4 excisional approaches in HCC? and how to decide btwn them?
Definition
open surgery, laparoscopic, RFA, Percutaneous Ethanol Injection (PEI).
no head-to-head trials choose by surgeon's expertise
Term
what is the maximal size of HCC lesion adequate for RFA?
Definition
3-4 cm
Term
2 obligations in the use of RFA for HCC?
Definition
1. size 3-4 cm maximum
2. proximity to portal triad - obstruction
Term
what is the maximal size of HCC lesion Tx with PEI?
Definition
3 cm
Term
what is the 5 yr tumor-free survival for a patient with HCC and Milan criteria who had underwent liver transplantation?
Definition
70%
Term
2 unknown facts about bridging Tx for patients waiting on the list for liver transplantation d/t HCC
Definition
1. if there is a survival benefit after transplantation
2. if recurrence rates should be calculated according to disease state post or pre bridging therapy
Term
what is an acceptable approach for downstaging HCC lesions for following trnasplantation which do not adhere to the Milan Criteria
Definition
TACE
Term
what is the TNM definition of stage 3 HCC
Definition
major vascular invasion
Term
what is the surgical approach to stage 3 HCC without Cirrhosis or child's A cirrhosis (2)
Definition
1. a small percentage of patients will achieve long-term survival, justifying an attempt at resection when feasible
2. downstaging with TACE can allow for liver transplantation
Term
what is the role systemic chemotherapy in the Tx of HCC?
Definition
none - no agent has shown any survival benefit
Term
what are the only two chemotherpeutic options administered via TACE have shown a survival benefit in HCC
Definition
Cisplatin and Doxorubicin
Term
how is regional chemotherapy (TACE) administered in patients with stage 3 or 4 HCC?
Definition
alongside with arterial embolization
Term
what are 6 possible adverse effects of the embolization therapy part of TACE in HCC patients
Definition
transient fever, abdominal pain, anorexia (all in >60% of patients, increased ascites, elevation of transaminases, cholecystitis
Term
The hepatic toxicities associated with embolization for the Tx of HCC may be ameliorated by the use of what
Definition
starch microspheres
Term
of the targeted new biological therapies for stage 3 and 4 HCC, which 2 regimens had shown a survival benefit?
Definition
1. Sorafenib (Nexavar)
2. bevacizumab (Avastin) plus erlotinib
Term
what is the Tx for Multiple unilobar tumors or tumor with vascular invasion
Definition
TACE or sorafenib (Nexavar)
Term
what is the TX for Bilobar tumors, with no vascular invasion
Definition
TACE with OLTX for patients with tumor response
Term
what is the Tx for Extrahepatic HCC or elevated bilirubin
Definition
sorafenib (Nexavar) or bevacizumab (Avastin) plus erlotinib (combination agent trials are in progress)
Term
what is a typical test for fibrolamellar HCC
Definition
neurotensin
Term
differences in fibrolamellar HCC compared with adult type
Definition
1. younger age (teenagers)
2. females more than males
3. Tx of choice is always surgical b/c poorer response to chemo
4. higher survival rates
5. no cirrhosis
6. less vascular invasion
Term
4 characteristics of epitheloid hemangioendothelioma
Definition
1. higher survival than adult type HCC
2. vascular origin as thus expresses factor 8
3. treated with liver transplantation
4. no cirrhosis
Term
what are 3 serologic markers used to follow up on the Tx of Cholangiocarcinoma
Definition
CEA, CA19-9, CA-125
Term
what 3 stains are positive in cholangiocarcinoma and which is negative?
Definition
cytokeratins 7, 8, and 19 and negatively for cytokeratin 20
Term
surgical Tx for hilar Cholangiocarcinoma - how many resectable, survival, procedure?
Definition
30% resectable, typical survival 24 months, bile duct resection and lymphadenectomy
Term
2 step Tx for distal Cholangiocarcinoma and survival?
Definition
1. resection of the extrahepatic bile ducts with pancreaticoduodenectomy
2. postoperative adjuvant radiotherapy (no survival benefit) or photodynamic therapy (shows survival benefit)
survival 24 months
Term
neoadjuvant radiotherapy with sensitizing chemotherapy followed by liver transplantation is indicated by the UNOS for perihilar cholangiocarcinoma, given 2 preconditions
Definition
1. lesion < 3 cm
2. no metastases
Term
what is the standard Tx for unresectable cholangiocarcinoma?
Definition
Gemcitabine + Cisplatin
Term
what is the only adjuvant Tx for cholangiocarcinoma that had shown a survival benefit?
Definition
photodynamic therapy
Term
what is the Px of Gallbladder carcinoma?
Definition
typical survival is 6 months in most cases, few cases are presented in stage 1 or 2 and entail higher much higher prognosis. prognosis is worse than HCC and CCC
Term
what is the Tx of unresectable gallbladder carcinoma?
Definition
chemo has no survival benefit
Term
what is the role of adjuvant radiation therapy in resectable gallbladder carcinoma?
Definition
none. no survival benefit
Term
2 characteristics of the patients with gallstone carcinoma
Definition
1. female
2. antecedent gallstone disease
Term
what is the MC clinical presentation of carcinoma of the ampulla of vater?
Definition
jaundice
Term
what is the definition of carcinoma of the ampulla of vater?
Definition
a tumor situated anywhere on the CBD within 2 cm of the ampulla
Term
what is the MC histologic type of carcinoma of the ampulla of vater?
Definition
adenocarcinoma
Term
what is the Tx of carcinoma of the ampulla of vater? what % of patients are resectable? what is the associated 5 yr survival?
Definition
pylorus-sparing pancreaticoduodenectomy, 80% resectable!, 25% if lymph node involvement and 50% if not
Term
what is the role of adjuvant chemo or radiotherapy?
Definition
none, no survival benefit
Term
which is the preferred method of adjuvant chemotherapy in colon cancer metastatic to the liver following metastasis resection?
Definition
systemic approach (vs. local infusion to the hepatic artery)
Term
what are the 3 benign types of tumor of the liver, which one is treated and why?
Definition
1. hemangioma
2. focal nodular hyperplasia
3. adenoma - Tx with resection b/c of 30% risk of bleeding and slightly elevated risk of HCC
Term
3 epidemiologic facts about esophageal carcinoma
Definition
1. blacks > whites
2. can be familial
3. associated with lower socioeconomic status
Term
what % of esophageal cancer is adenocarcinoma vs. SCC in the western world today?
Definition
70%
Term
9 risk factors for esophageal SCC
Definition
hot tea, smoking, alcohol, nitrites, smoked opiates, lye (used for making soap), achalasia, fungal toxins in canned vegetables, radiation
Term
which 2 syndromes are associated with increased risk of esophageal SCC?
Definition
1. plammer-vinison
2. tylosis palmaris et plantaris - congenital hyperkeratosis and pitting of the palms and soles
Term
which 3 nutritional deficiencies are associated with an increased risk of esophageal SCC?
Definition
zinc, selenium, and vitamin A
Term
2 risk factors for esophageal adenocarcinoma except barret's (GERD)
Definition
bisphosphonates, obesity
Term
which medical condition is associated with and increased risk of esophageal SCC
Definition
head and neck malignancy
Term
6 sites of metastases in esophageal cancer
Definition
adjacent and supraclavicular lymph nodes, liver, lungs, pleura, and bone
Term
paraneoplastic phenomenon in esophageal SCC and its mechanism
Definition
hypercalcemia d/t PTHrp
Term
what are the methods used in the Dx and workup of esophageal cancer?
Definition
endoscopy (Bx, brush smears), abdominal and chest CT or EUS, PET-CT
Term
what is the use of EUS in the workup of esophageal cancer
Definition
assessment of mediastinum and para-aortic lymph nodes
Term
what is the role of PET-CT in the workup of esophageal cancer?
Definition
assessment of resectability - spread to mediastinal lymph nodes
Term
what is the 5 yr survival for patients with esophageal cancer
Definition
5%
Term
what % of esophageal cancers are resectable at presentation
Definition
50%
Term
what is the 5 yr survival for esophageal cancer undergoing resection?
Definition
20%
Term
what is the perisurgical mortality rate in esophageal cancer? what are the causes
Definition
5%, fistulas/respiratory complications and subphrenic abscesses
Term
what is the survival rate for primary radiation therapy for esophageal cancer as a substitute for surgical resection? what is the advantage and the downside?
Definition
same survival rates as for surgery (20% 5yr), spares the perioperative mortality, doesn't palliate as efficiently
Term
what is the Tx of choice for esophageal cancer for patients with a good performance status?
Definition
chemoradiation +/- resection depending on residual disease after chemoRx
Term
what is the most efficient chemo for the Tx of esophageal cancer
Definition
a combination of cisplatin and another drug
Term
4 methods of palliation in esophageal cancer
Definition
1. dilation endoscopy
2. laser
3. metal stent
4. surgical gastrostomy/jejunostomy
Term
which condition is associated with both esophageal adenocarcinoma and SCC?
Definition
celiac sprue
Term
incidence of gastric adenocarcinoma is decreasing or increasing worldwide?
Definition
decreasing
Term
risk factor for gastric adenocarcinoma
Definition
low socioeconomic class
Term
how does migrating from a high incidence area of gastric adenocarcinoma to a low incidence area affect the risk of disease
Definition
in the adult in doesn't change the risk in its offsprings the risk is reduced, suggesting an early life environmental factor, possibly nutritional
Term
4 histologic types of gastric cancer and their incidence
Definition
85% - adenocarcinoma
15% - lymphoma, GIST, leimyosarcoma
Term
5 characteristics of diffuse type gastric adenocarcinoma in comparison to intestinal type
Definition
1. lintis plastica
2. decreased distensibility
3. involves any part of the stomach
4. poorer prognosis
5. younger patients
Term
why is lintis plastica formed in diffuse type gastric adenocarcinoma
Definition
loss of expression of E-cadherin, cell don't attach to each other to form a mass
Term
3 characteristics of intestinal type gastric adenocarcinoma in comparison to intestinal type
Definition
1. create ulcerative lesions
2. antrum and lesser curvature
3. prolonged precancerous H.Pylori
Term
what is the hypothesis for the etiology of gastric adenocarcinoma? exogenous and endogenous factors that contribute to it?
Definition
lower socioeconomic class patients ingest decayed food containing bacteria able to turn nitrates in salty, smoked, food into nitrites which are carcinogenic.
factors that are associated with an increased risk of attaining such bacteria: low gastric acidity, H2 blockers, prior gastric surgery, pernicious anemia/atrophic gastritis
Term
which blood type is associated with higher risk of gastric adenocarcinoma
Definition
A
Term
which disease is associated with higher risk of gastric adenocarcinoma?
Definition
Ménétrier's disease - extreme hypertrophy of gastric rugal folds
Term
what kind of mutation is associated with a significant increased risk of gastric adenocarcinoma
Definition
germ line mutation in the E-cadherin gene CDH1
Term
what genetic alterations have been associated with invasive disease in gastric adenocarcinoma
Definition
epigenetic changes - methylation
Term
what gene mutations are associated in the carcinogenesis of gastric adenocarcinoma (except for E-cadherin)
Definition
K-ras, p53, APC, DCC (deleted in colon cancer)
Term
which protein is associated with progression from dysplasia to gastric adenocarcinoma
Definition
Cyclin E
Term
which protein can be found in the nucleus of tumor cells at the leading edge of invasion in gastric adenocarcinoma
Definition
beta-catenin
Term
4 unique sites of metastasis given names in gastric adenocarcinoma
Definition
blumer's shelf - peritoneal cul-de-sac
sister mary joseph - periumbilical
kruckenberg - ovary
virchow's node - left supraclavicular
Term
what is the MC site of hematogenous spread in gastric adenocarcinoma?
Definition
liver
Term
4 Unusual clinical features associated with gastric adenocarcinomas
Definition
migratory thrombophlebitis, microangiopathic hemolytic anemia, diffuse seborrheic keratoses (so-called Leser-Trélat sign), and acanthosis nigricans.
Term
what is the first test in the workup of a patient suspected of gastric adenocarcinoma
Definition
double contrast radiography
Term
4 factors that influence Px after resection in gastric adenocarcinoma
Definition
extent of wall invasion, regional lymph node involvement, vascular invasion, and abnormal DNA content (i.e., aneuploidy)
Term
what % of patient are eligible for complete resection including regional lymph nodes in gastric adenocarcinoma?
Definition
30%
Term
which location is associated with a higher survival rate post resection, proximal or distal gastric adenocarcinoma
Definition
distal
Term
what is the role of complete resection (includes regional lymph node resection)?
Definition
it is the only Tx that offers a chance for cure, but it is not associated with higher survival and is associated with more post-op morbidity
Term
what are the only 2 CI to resection of primary tumor in gastric adenocarcinoma and why?
Definition
ascites or extensive hepatic or peritoneal metastases
decreasing tumor bulk is the best palliative Tx
Term
what is the role of radiation therapy in gastric adenocarcinoma and why?
Definition
palliation only. the tumor is radioresistant
Term
in which way is adjuvant chemotherapy administered in gastric adenocarcinoma?
Definition
perioperative - before and after surgery
Term
2 chemo regimens given in advanced gastric adenocarcinoma
Definition
1. cisplatin and epirubicin
2. taxel and 5FU or irinotecan
Term
what is the MC extranodal site for lymphoma?
Definition
stomach
Term
what is the MC histologic type of gastric lymphoma
Definition
non hodgkin's B cell lymphoma
Term
what is the 1st line Tx of gastric lymphoma and why?
Definition
Abx to eradicate H.Pylori b/c this leads to regression of disease in 75% of cases
Term
what is the underlying genetic abnormality in gastric lymphoma resistant to H.Pylori eradication?
Definition
t(11:18)
Term
what is the Tx of localized high grade gastric lymphoma?
Definition
surgical plus adjuvant chemo leading to a 5 yr survival of 50%
Term
what is the Tx of gastric lymphoma with preoperative radiographic evidence of nodal involvement?
Definition
chemo - R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone
Term
what is the role of adjuvant radiotherapy in gastric lymphoma?
Definition
none, b/c most recurrences are not at tumor bed but distant
Term
what is the MC of gastric sarcomas
Definition
fundus
Term
after diagnosing gastric sarcoma, what should be the first next step?
Definition
checking for c-kit mutation b/c 50% of patients with gastric GIST are responsive to Gleevec
Term
what is the 2nd line Tx for patients with gastric GIST that are unresponsive to Gleevec
Definition
Sunitinib (Sutent)
Term
what is the role of chemo in the Tx of gastric GIST?
Definition
none, GIST are unresponsive to chemo
Term
what is the Tx of choice in gastric sarcoma (non c-kit)?
Definition
surgical
Term
what % of polyps become malignant?
Definition
less than 1%
Term
what % of polyps bleed?
Definition
less than 5%
Term
colon cancer develops more frequently in pedunculated or sessile polyps?
Definition
sessile
Term
what is the histology most associated with colon cancer in polyps?
Definition
villous adenoma
Term
in colon, what is the size of an adenoma that holds the most substantial risk of being malignant?
Definition
over 2.5 cm
Term
after finding a polyp why should the whole colon be checked?
Definition
synchronous lesions will appear in one third of the cases
Term
after finding and removing a polyp from the colon why should colonoscopy be repeated periodically
Definition
50% chance of recurrence and a higher than standard risk of colorectal cancer
Term
what are 3 dietary factors that affect the risk of colorectal cancer?
Definition
animal fat, high caloric intake, high cholesterol levels
Term
what is the role of a high vegetable and fruit diet in preventing colorectal cancer
Definition
none, diet high in fiber has failed to show effect on the risk of colorectal cancer
Term
mortality from colorectal cancer is associated with mortality from what other disease
Definition
coronary artery disease
Term
what are 6 general risk factors for colorectal cancer
Definition
diet, hereditary syndromes, IBD, strep bovis, ureterosigmoidostomy, tobacco
Term
what % of colorectal patients have a family history of the disease
Definition
25%
Term
what 6 lesions are associated with Gardner's syndrome?
Definition
Osteomas, fibromas, lipomas, epidermoid cysts, ampullary cancers, congenital hypertrophy of retinal pigment epithelium
Term
what 2 lesions are associated with HNPCC
Definition
Endometrial and ovarian tumors
Term
what 4 malignancies are associated with Peutz-Jegher's syndrome?
Definition
ovary, breast, pancreas, endometrium
Term
what is the first Tx once FAP has been diagnosed?
Definition
total colectomy
Term
2 possible surveillance methods of the offsprings of patients with FAP?
Definition
1. annual sigmoidoscopy from age 15-35
2. APC mutated gene in mononuclear cells in the peripheral blood
Term
what are the 3 amsterdam criteria for HNPCC
Definition
1. 3 or more relatives with CRC where one of the patients is a 1st degree relative of the other two
2. the disease spans at least two generations
3. at least one patient is under 50
Term
what is the MC site of tumor in HNPCC?
Definition
proximal colon
Term
what is the histology and prognosis of CRC in HNPCC compared with sporadic CRCs?
Definition
despite being poorly differentiated in histology it has better prognosis
Term
what is the recommended screening strategy in females afflicted with HNPCC?
Definition
biannual colonoscopy (every 2 years), intermittent pelvic US and endometrial Bx
Term
what are the 2 genes mutated in HNPCC and their role
Definition
hMLH1 and hMSH2 - DNA mismatch repair when damaged causeing microsatellite instability
Term
what is the rate at which the risk of CRC is increased in people with UC and at what point of time?
Definition
10 yrs into the IBD the risk of CRC climbs to a rate increase of 1% every year
Term
what % of patients with IBD will develop CRC after age 25
Definition
8-30%
Term
who are the IBD patients more prone to suffer from CRC?
Definition
those with pancolitis
Term
what is the role of colonoscopic screening for CRC in patients with IBD?
Definition
controversial, it has not shown survival benefit
Term
what is the primary prophylactic Tx of choice for IBD patients experiencing frequent bouts of disease for over 15 yrs?
Definition
total colectomy
Term
5 nutritional supplementations and drugs proven effective for primary and secondary prevention of CRC
Definition
aspirin, NSAIDs, estrogen replacement therapy, folic acid, oral calcium
Term
what are the 2 options the american cancer society recommendations provide for CRC screening
Definition
1. annual occult fecal blood and sigmoidoscopy every 5 yrs as of age 50
2. colonoscopy every 10 yrs as of age 50
Term
what % of CRCs are detected on occult fecal blood?
Definition
50%
Term
staging of CRC
Definition
1. stage I (T1–T2,N0,M0) - doesn't pass after muscularis
2. stage II (T3,N0,M0) - passes all the way through muscularis
3. stage III (TX,N1,M0) - regional lymph involvement
4. stage IV (TX,NX,M1) - metastases
Term
why is the 5 yr survival rate a good predictor of cure in CRC
Definition
most recurrences occur within 4 yrs of resection
Term
what should be the minimum number of nodes examined when staging CRC and why?
Definition
12. whether the number of lymph nodes involved is over 3 or under 4 impedes greatly on Px
Term
10 predictors of poor Px after resection in CRC
Definition
regional lymph node involvement, number of lymph nodes involved, tumor penetration through the bowel wall, perforation, spread to adjacent organs, presurgery elevation in CEA, aneupleudy, certain mutations such as deletion of 18 chromosome (DCC), venous invasion, poorly differentiated histology
Term
In contrast to most other cancers, the prognosis in colorectal cancer is not influenced by?
Definition
the size of the primary lesion
Term
what is the MC organ for metastases in CRC
Definition
liver
Term
which mutation detected is associated with a better outcome in CRC
Definition
APC - microsatellite instability
Term
4 tests that should be preformed preoperatively in CRC?
Definition
LFTs, CEA, CXR, colonoscopy (if possible)
Term
why is it so important to perform colonoscopy preoperatively and when should it be done if it not possible to do it before resection
Definition
there is a likelihood of synchronous disease somewhere else in the colon. should be done several months post-operatively
Term
what is the follow up of patients with CRC who have undergone complete resection
Definition
1. semi annual physical examination and annual LFTs or 3 monthly CEA only
2. CT or colonoscopy every 3 years
Term
why is it important to repeat colonoscopy after 3 years of disease
Definition
the chance of suffering a second bowel malignancy is 5% and adenoma - 15%
Term
2 roles of chemoradiation in CRC
Definition
1. adjuvant Tx to reduce recurrence rates
2. neoadjuvant Tx to reduce primary tumor size and allow resection
Term
what is the effect of radiation therapy in CRC on survival?
Definition
none
Term
adjuvant chemoradiation is indicated in what stages of CRC
Definition
2-3
Term
what are 2 optional 1st line Tx regimens in metastatic CRC?
Definition
1. FOLFIRI +/- Cetuximab (Erbitux)
2. FOLFOX
Term
what are 2 optional 2nd line Tx regimens in metastatic CRC?
Definition
1. alternate choice btwn FOLFIRI/FOLFOX
2. Cetuximab (Erbitux) or panitumumab (Vectibix) - EGFR inhibitors
Term
in what subset group of patients with CRC are EGFR inhibitors - Cetuximab (Erbitux) and panitumumab (Vectibix) - ineffective?
Definition
patients with a K-ras mutation
Term
what is the major side effect of EGFR inhibitors (Cetuximab (Erbitux) and panitumumab (Vectibix)) and what is this an indication for?
Definition
rash. indicating good therpeutic response
Term
what is the most effective adjuvant chemo Tx option for stage 3 CRC?
Definition
FOLFOX (FOLFIRI or Cetuximab do not show survival or recurrence benefit)
Term
what is the place for adjuvant chemotherapy in stage 2 CRC?
Definition
usually it is not beneficial except in the following 3 cases: perforated tumor, Ty lesions, lymphovascular invasion
Term
what is an adjuvant method to increase survival and decrease recurrence in rectal CRC stage 2/3?
Definition
neo/adjuvant 5FU+radiation therapy
Term
in what 3 situations should small bowel tumors be taken into account in the DD
Definition
1. recurrent bouts of bowel obstruction w/o prior surgery or IBD
2. intussuception in the adult
3. evidence of bleeding with negative findings on endoscopy
Term
what is the diagnostic procedure of choice for suspected small bowel tumor
Definition
small bowel barium study with enteroclysis (NGT to constantly insert contrast)
Term
what is the role of clinical and radiological findings in determining whether a small bowel tumor is benign or malignant?
Definition
none
Term
4 benign tumors of the small bowel
Definition
lipomas, angiomas, adenomas, leiomyomas
Term
MC malignant tumor of the small bowel
Definition
adenocarcinoma - 50%
Term
3 risk factors for small bowel malignancy
Definition
celiac, AIDS, chronic enteritis
Term
what is the MC site of adenocarcinoma in the small bowel?
Definition
distal duodenum-proximal jejunum
Term
Tx of small bowel adenocarcinoma
Definition
resection
Term
Lymphoma in the small bowel may be primary or secondary. Dx is histologic in a clinical setting that is absent of the following
Definition
adenopathy, hepatosplenomegaly, no evidence on CXR, CT scan, or blood smear or on bone marrow Bx
Term
4 step workup needed to confirm Dx of primary small bowel lymphoma
Definition
1. Bx of bowel lesion
2. CXR
3. CT
4. BM Bx or blood smear
Term
what is the incidence of small bowel lymphoma amongst the malignancies of the small bowel?
Definition
20%
Term
what are the histologic characteristics of primary lymphoma of the small bowel?
Definition
diffuse, large cell, T cell
Term
3 sites of lymphoma in the small bowel by decreasing order of incidence and the reason for this
Definition
ileum, jejunum, duodenum - decreasing order of lymphatic component
Term
3 risk factors for lymphoma of the small bowel (similar to the ones for small bowel malignancy)
Definition
celiac, chronic enteritis, immune deficiency from any reason
Term
what are the rates of cure in small bowel lymphoma?
Definition
75% in resectable disease and 25% in unresectable disease
Term
what is immunoproliferative small intestinal disease (IPSID), Mediterranean lymphoma, or heavy chain alpha disease disease
Definition
diffuse lymphoma involving the entire bowel
Term
what cell type of lymphoma is IPSID?
Definition
B cell
Term
what are 2 unique findings in IPSID?
Definition
1. clubbing
2. the presence in the blood and intestinal secretions of IgA with a shortened alpha heavy chain and devoid of light chains
Term
what is the Tx of IPSID?
Definition
Tetracyline + chemo
Term
where are carcinoid tumors found in the gut
Definition
remenants of the midgut - distal duodenum to ascending colon. MC site is distal ileum near ileocecal valve
Term
what is the incidence of anal carcinomas among large bowel malignancies?
Definition
1%
Term
2 histologic types of anal carcinoma according to their anatomical origin?
Definition
1. basaloid or cuboidal - proximal to the pectinate line 40%
2. SCC distal to the pectinate line 60%
Term
what is the premalignant lesion of anal carcinoma?
Definition
condylomata acuminata caused by HPV
Term
what is the Tx of choice for patients with anal carcinoma
Definition
chemoradiation
Term
what are the rates of disappearance of tumor smaller than 3 cm for chemoradiation in anal carcinoma
Definition
over 80%
Term
what is the rate of recurrence for patients achieving complete remission after chemoradiation for anal carcinoma
Definition
under 10%
Term
what are the rates of cure with chemoradiation for anal carcinoma
Definition
70%
Term
what % of breast cancers can be associated to a germ-line mutation and what are 4 known such mutations?
Definition
10%, BRCA1, BRCA2, Li-Fraumeni syndrome, PTEN
Term
what is the lifetime risk of a female with BRCA1 to develop breast cancer?
Definition
60-80%
Term
what are the malignancies associated with male BRCA1 carrier
Definition
prostate and breast
Term
in what % of sporadic breast cancer is the acquired mutation in p53 present? PTEN?
Definition
40%, 10%
Term
the oncogene erbB2 (HER/2 neu) is present in what % of breast cancers?
Definition
25%
Term
what is the risk of a female who went through menarch at the age of 16 to develop breast cancer in comparison to a female who went through menarch at the age of 12?
Definition
50%
Term
menopause that occurs 10 yrs before the median age (52) reduces the risk of breast cancer by what %
Definition
35%
Term
what is the breast cancer risk reduction for females that give birth before the age of 18 compared with nulliparus females?
Definition
35%
Term
breast cancer risk factors
Definition
age at menarch, menopause, first full term pregnancy, increased height and weight, no breast feeding,increased caloric intake, moderate alcohol intake, OCP, exposure to radiation before the age of 30
Term
why is increased caloric intake associated with and increased risk of breast cancer?
Definition
earlier menarch, later menopause, higher levels of estrogen post-menopause
Term
what is the role of folic acid in reduction of breast cancer risk?
Definition
it reduces the risk in alcohol drinkers only
Term
which medication is known to reduce the risk of breast cancer?
Definition
low dose aspirin
Term
what is the effect of OCP on the risk of breast cancer, endometrial cancer and ovarian cancer
Definition
it slightly elevates the risk of breast cancer and decreases the risk of both ovarian and endometrial cancer
Term
what is the effect of HRT on colorectal cancer?
Definition
reduces the risk
Term
what is the overall effect of HRT on the risk of suffering and adverse event?
Definition
positive
Term
when you find a dominant breast mass in a premenopausal female, what is the next step in the workup?
Definition
aspiration
Term
when you find a mass breast mass in a post-menopausal female, what is the next step in workup?
Definition
aspiration
Term
when you find a breast mass that is questionable in a pre-menopausal female, what is the next step in workup?
Definition
perform PE again during the follicular phase of the period?
Term
in the workup of a breast mass that turns out to be solid on aspiration, what is the next step in workup?
Definition
mammogram
Term
in a solid breast mass suspicious of being malignant on mammogram, what is the next step in workup
Definition
Bx
Term
in the workup of a breast cyst, if the fluid aspirated is non-bloody, what is the next question you ask yourself?
Definition
is there a residual mass
Term
in the workup of a breast cyst, if the fluid aspirated is non-bloody and there's a residual mass, what is the next course of action?
Definition
mammogram and Bx
Term
in the workup of a breast cyst, if the fluid aspirated is non-bloody and there's no residual mass, what is the next course of action?
Definition
check if fluid reaacumulates
Term
in the workup of a breast cyst, if the fluid aspirated is non-bloody and there's no residual mass but fluid reaccumulates, what is the next course of action?
Definition
reaspirate and check if fluid reaccumulates again
Term
in the workup of a breast cyst, if the fluid aspirated is non-bloody and there's no residual mass but fluid reaccumulates after 2 aspirations, what is the next course of action?
Definition
mammogram and Bx
Term
what is the triple diagnosis technique in a breast lesion? if it's triple negative what is the false negative?
Definition
PE, mammogram, FNA
Term
what do you do with a nonpalpable mammographic lesion that has a low index of suspicion?
Definition
mammographic follow up in 3-6 months
Term
what is the workup of a breast mass in a pregnant female?
Definition
same as in the non-pregnant female
Term
what is the risk of breast cancer in females who have had a Bx of a breast mass that was benign in comparison to those who haven't?
Definition
risk is elevated
Term
what is the risk of breast cancer in patients who have had a Bx of ductal/lobular hyperplasia or atypical hyperplasia in comparison to females who have not had a Bx at all? and what if these females had a 1st degree relative with breast cancer?
Definition
X4, X9
Term
what is the risk reduction of mortality in patients over the age of 50 who perform screening for breast cancer?
Definition
25%
Term
which 3 populations are recommended to perform breast cancer screening with MRI before the age of 40?
Definition
1. radiation to chest ages 10-30
2. BRCA1/2 carriers or unknown 1st degree relatives of breast cancer patients/carriers
3. Li Fraumeni, Bannayan, Cowden syndromes
Term
what is the recommend age to start screening for breast cancer with mammogram?
Definition
40
Term
in the staging of breast cancer, what is T0
Definition
no tumor
Term
in the staging of breast cancer, what is Tis
Definition
carcinoma in situ
Term
in the staging of breast cancer, what is T1
Definition
lesion smaller than 2 cm
Term
in the staging of breast cancer, what is T1a
Definition
lesion 0.1-0.5 cm
Term
in the staging of breast cancer, what is T1b
Definition
lesion 0.5-1 cm
Term
in the staging of breast cancer, what is T1c
Definition
lesion 1-2 cm
Term
in the staging of breast cancer, what is T2
Definition
lesion 2-5 cm
Term
in the staging of breast cancer, what is T3
Definition
lesion larger than 5 cm
Term
in the staging of breast cancer, what is T4
Definition
lesion expands to chest wall, inflammation, satellite lesions, ulcerations
Term
in the staging of breast cancer, what is PN0(i-)
Definition
No regional lymph node metastasis histologically, negative IHC
Term
in the staging of breast cancer, what is PN0(i+)
Definition
No regional lymph node metastasis histologically, positive IHC, no IHC cluster greater than 0.2 mm
Term
in the staging of breast cancer, what is
PN0(mol-)
Definition
No regional lymph node metastasis histologically, negative molecular findings (RT-PCR)
Term
in the staging of breast cancer, what is
PN0(mol+)
Definition
No regional lymph node metastasis histologically, positive molecular findings (RT-PCR)
Term
in the staging of breast cancer, what is
PN1
Definition
Metastasis in one to three axillary lymph nodes, or in internal mammary nodes with microscopic disease detected by sentinel lymph node dissection but not clinically apparent
Term
in the staging of breast cancer, what is
PN1mi
Definition
Micrometastasis (>0.2 mm, none >2 mm)
Term
in the staging of breast cancer, what is
PN1a
Definition
Metastasis in one to three axillary lymph nodes
Term
in the staging of breast cancer, what is
PN1b
Definition
Metastasis in internal mammary nodes with microscopic disease detected by sentinel lymph node dissection but not clinically apparent
Term
in the staging of breast cancer, what is
PN1c
Definition
Metastasis in one to three axillary lymph nodes and in internal mammary lymph nodes with microscopic disease detected by sentinel lymph node dissection but not clinically apparent.a (If associated with greater than three positive axillary lymph nodes, the internal mammary nodes are classified as pN3b to reflect increased tumor burden.)
Term
in the staging of breast cancer, what is
PN2
Definition
Metastasis in four to nine axillary lymph nodes, or in clinically apparent internal mammary lymph nodes in the absence of axillary lymph node metastasis
Term
in the staging of breast cancer, what is
PN3
Definition
Metastasis in 10 or more axillary lymph nodes, or in infraclavicular lymph nodes, or in clinically apparenta ipsilateral internal mammary lymph nodes in the presence of 1 or more positive axillary lymph nodes; or in more than 3 axillary lymph nodes with clinically negative microscopic metastasis in internal mammary lymph nodes; or in ipsilateral subcarinal lymph nodes
Term
in breast cancer what is stage 0
Definition
TisN0M0
Term
in breast cancer what is stage 1
Definition
T1N0M0
Term
in breast cancer what is stage 2a
Definition
T0N1M0 or T1N1M0 or T2N0M0
Term
in breast cancer what is stage 2b
Definition
T2N1M0 or T3N0M0
Term
in breast cancer what is stage 3a
Definition
T0N2M0 or T1N2M0 or T2N2M0 or T2N1,2M0
Term
in breast cancer what is stage 3b
Definition
T4anyNM0 or anyTN3M0
Term
6 characteristics of Luminal A subtype according to gene expression breast cancer
Definition
1. favorable prognosis
2. cytokeratin 8 and 18
3. resistant to chemotherapy
4. responsive to endocrine therapy
5. highest ER+ levels
6. low-grade
Term
what is the prognosis for Luminal B (according to gene expression) breast cancer in comparison to A?
Definition
worse
Term
definition of normal-breast-like subtype of breast cancer and its Px
Definition
gene expression resembles that of normal breast tissue. Px is similar to Luminal B
Term
what is the gene expression subtype associated with BRCA related breast cancers
Definition
Basal/triple negative disease
Term
Basal subtype breast cancer are of what grade?
Definition
high grade
Term
which markers does Basal/triple negative breast cancer express?
Definition
cytokeratins 5/6 and 17, vimentin, p63, CD10, α-smooth muscle actin, (EGFR)
Term
5 CI for lumpectomy in breast cancer
Definition
tumors >5 cm, involving the nipple, extensive intraductal disease involving multiple quadrants of the breast, history of collagen-vascular disease, no access to radiation therapy
Term
3 indications for radiation therapy of the breast post mastectomy
Definition
T2 in size, positive margins, positive nodes
Term
3 predictors of recurrence of breast cancer in the surgical bed?
Definition
lymph node, vascular involvement, extensive intraductal component
Term
what is the effect on survival rates for recurrence in the breast of patients who have undergone breast conserving surgery in comparison to those who underwent mastectomy
Definition
recurrence is associated with poorer survival
Term
what are the rates of recurrence in the surgical bed after successful breast conserving surgery?
Definition
less than 10%
Term
what is the prognostic significance of the hormone receptors in breast cancer?
Definition
patients who are negative in both ER and PR or in either one of them, are more likely to have recurrence of disease
Term
what is the most significant prognostic factor in breast cancer
Definition
tumor staging
Term
the decision to administer adjuvant chemotherapy is sometimes hard to determine. (for example: 1 cm tumor, negative lymph nodes, HER2+) what is a tool to decide?
Definition
Oncotype - 21 genes that classify the patient to either low, intermediate or high risk
Term
what are the prognostic factors in breast cancer?
Definition
stage, grade, molecular biology (HER2), microvascular proliferation, certain genes present in oncotype, collagenase, cathepsin D, plasminogen activator, plasminogen activator receptor, and the metastasis-suppressor gene nm23
Term
what adjuvant Tx is given to females who are premenopausal and have positive lymph nodes (per Bx)
Definition
Multidrug chemotherapy + tamoxifen if ER-positive + trastuzumab in HER2/neu–positive tumors
Term
which 3 premenopausal female populations with breast cancer receive adjuvant chemotherapy?
Definition
1. positive lymph nodes
2. T >2 cm,
3. T 1–2 cm with other poor prognostic variables
Term
what hormonal Tx is given to postmenopausal females? which post menopausal females receive hormonal Tx?
Definition
ER+, tamoxifen + aromatase inhibitors
Term
in what population of females is adjuvant chemotherapy for breast cancer controversial?
Definition
postmenopausal females with ER+ receptors
Term
when do breast cancer recurrence occur?
Definition
over 50% occur more than 5 yrs after initial Tx
Term
what is the workup for a patient suspected of breast cancer metastasis?
Definition
Bx - must be done to avoid mistakes with other conditions (TB, MM, sarcoidosis)
Term
4 conditions unlikely to respond to hormonal Tx despite hormone positive breast cancer?
Definition
short disease-free intervals, rapidly progressive visceral disease, lymphangitic pulmonary disease, or intracranial disease
Term
which subset of patients with metastatic breast cancer should receive anti-hormonal therapy?
Definition
all metastatic patients
Term
what should be the initial anti hormonal therapy for metastatic breast cancer?
Definition
aromatase inhibitors
Term
which subset of patients with metastatic breast cancer has a higher respoonse rate to aromatase inhibitors than to tamoxifen?
Definition
HER2+, ER+
Term
what are 3 anti hormonal therapies used solely in pre-menopausal females?
Definition
surgery, castration, LHRH agonists
Term
what is the place of surgical adrenalectomy in metastatic breast cancer hormonal Tx?
Definition
rarely employed 2nd line Tx
Term
what is Fulvestrant in the hormonal Tx of metastatic breast cancer?
Definition
an estrogen receptor blocker with no estrogen agonist effect at all, used as 3rd line Tx
Term
what is the MC used 4th line hormonal Tx in metastatic breast cancer?
Definition
high-dose progesterones - given after AIs, tamoxifen, fulvestrant
Term
what is an alternative 4th line hormonal Tx in metastatic breast cancer?
Definition
additive androgens/estrogens
Term
what are 3 surgical options targeted at anti-hormonal effects in metastatic breast cancer?
Definition
hypophysectomy, adrenalectomy, castration
Term
in what combinations should anti hormonal Tx for metastatic breast cancer be given?
Definition
no combination. only monotherapy and never alongside chemotherapy
Term
what is the standard adjuvant chemo combination given in breast cancer?
Definition
CMF - cyclophosphamide, MTX, Fluorouracil
Term
what is the 1st line chemotherapy for metastatic breast cancer?
Definition
a combination of drugs from 4 groups: alkylating agents, taxanes, antimetabolites, anthracyclines
Term
what is the 2nd line chemotherapy for metastatic breast cancer?
Definition
the approach is to use monotherapy: paclitaxel or anthracycline
Term
which drug can increase the response rate and duration achieved by paclitaxel for the 2nd line Tx of metastatic breast cancer?
Definition
bevacizumab (Avastin)
Term
what is another line of chemotherapy after initial and monotherapy for metastatic breast cancer?
Definition
high dose chemo with autologous BMT
Term

what is a common Tx approach for stage 3 - locally advanced disease - breast cancer?

stage 3 = N2-N3 with any T and M0 

Definition
neoadjuvant chemotherapy that include an Anthracycline. this allows inoperable disease to become operable or susceptible to radiation therapy
Term
what is the long term disease free survival in patients with locally advanced breast cancer treated with neoadjuvant chemo?
Definition
50%
Term
in a female who had suffered breast cancer, what is the risk of developing cancer in the contralateral breast?
Definition
0.5% per year
Term
2 positive effects of long term use of tamoxifen for the prevention of breast cancer?
Definition
increased bone mineralization, reduction in cholesterol levels
Term
what is the risk of uterine cancer after 5 yrs of using tamoxifen as breast cancer prevention
Definition
0.75%
Term
what is and adverse effect of tamoxifen for the prevention of breast cancer, besides endometrial cancer?
Definition
increased rate of cataracts
Term
3 points of comparison btwn tamoxifen and Raloxifen in the prevention of breast cancer?
Definition
1. same efficacy for invasive breast cancer but reduced efficacy for Raloxifene in the prevention of non-invasive disease
2. reduced thromboembolic events with Raloxifene
3. reduced rates of endometrial cancer with raloxifene
Term
what % of patients with untreated DCIS develop invasive breast cancer within 5 yrs?
Definition
33%
Term
what is the 3 fold management of breast DCIS?
Definition
lumpectomy, radiation therapy, tamoxifen
Term
5 prognostic features that indicate high risk of local recurrence in DCIS (and may aid in the decision of whether to pursuit full Tx)
Definition
age<40, extensive DCIS, histologic findings such as necrosis, comedo subtype with HER2, poor nuclear grading
Term
what is the role of sentinel lymph node dissection in DCIS breast cancer?
Definition
not needed unless there is the slightest evidence of invasion, even micrometastasis
Term
what % of patients with LCIS develop invasive carcinoma within 20 yrs
Definition
30%, usually invasive ductal carcinoma
Term
what is the Tx of LCIS of the breast
Definition
local excision, SERMs for 5 yrs, annual mammogram follow up
Term
is locally advanced breast cancer more common in males or females?
Definition
males, less tissue
Term
When male breast cancer is matched to female breast cancer by age and stage, what can be said about the prognosis
Definition
same
Term
how does gyncecomastia affect the risk of breast cancer in the male?
Definition
increases it
Term
what is the Tx of male breast cancer?
Definition
mastectomy and axillary node dissection= modified radical mastectomy
Term
which male patients with breast cancer should receive radiation therapy?
Definition
locally advanced disease or positive nodes
Term
what % of male patients with breast cancer are positive for estrogen receptors?
Definition
90%
Term
what % of male metastatic breast cancer are responsive to hormonal therapy
Definition
60%
Term
what are the guidelines for adjuvant systemic Tx for male breast cancer?
Definition
same as for female
Term
what is the effect of early detection of breast cancer recurrence on the survival?
Definition
none
Term
what is the recommended follow up regime for breast cancer
Definition
1. Hx and PE every 3-6 months for the 1st 3 yrs, then every 6-12 months for another 2 yrs and then every yr
2. monthly breast self examination
3. annual mammogram
4. annual pelvic examination
Term
what should be avoided in the follow up of breast cancer?
Definition
CBC, chemistries, markers, CXR, CT, bone scan, liver US
Term
what % of lung cancers occur in never smokers or former smoker (quit over a year ago)?
Definition
60%
Term
what is the number of never smoker amongst lung cancer patients?
Definition
1 in 5 females, 1 in 12 males
Term
what is the overall lifetime probability to develop lung cancer?
Definition
8% in males and 6% in females
Term
what is the relative risk of lung cancer for people who currently smoke in comparison to those who never smoked?
Definition
20 fold
Term
what is the relative risk of lung cancer for people who used to smoke in comparison to those who never smoked?
Definition
9 fold
Term
what % of lung cancer are associated with smoking?
Definition
90%
Term
what is the effect of increased duration of quitting to smoke on the risk of lung cancer?
Definition
the risk is reduced but is never equal to the non-smoker
Term
what is the % increase in risk of never smokers who have been married many years to smokers?
Definition
20-30%
Term
risk factors other than smoking for lung cancer
Definition
asbestos, arsenic, bischloromethyl ether, hexavalent chromium, mustard gas, nickel, polycyclic aromatic hydrocarbons, low fruit and vegetable intake during adulthood, Prior lung diseases such as chronic bronchitis, emphysema, and tuberculosis, radiation, radon
Term
what is the effect of retenoids and carotenoids in smokers on the risk of lung cancer?
Definition
increased (though was expected to be protective)
Term
what is the reduction in risk of lung cancer with the cessation of smoking before middle age?
Definition
90%
Term
what is the effect of smoking cessation on the already ill lung cancer patient?
Definition
elevated survival, decreased adverse effects from Tx and better quality of life
Term
what is the most effective drug for the cessation of smoking
Definition
verenicline - nicotinic acetylcholine partial agonist
Term
what are 1st line drugs for cessation of smoking
Definition
verenicline and bupropion
Term
what is a major adverse effect of both verenicline and bupropion for the cessation of smoking?
Definition
suicidal ideation
Term
what are 2nd line drugs for cessation of smoking
Definition
clonidine and nortryptiline
Term
what are 3 notable germ line mutations that increase the risk of lung cancer?
Definition
polymorphism in P450 CYP1A1
Li Fraumeni syndrome (p53)
Retinoblastoma (RB)
Term
what is the increase in risk for 1st degree probands of patients with lung cancer?
Definition
X3
Term
what are the 4 major epithelial subtypes of lung cancer?
Definition
SCLC
NSCLC: adenocarcinoma, SCC, large cell
Term
adenocarcinoma is the MC histologic subtype of lung cancer in which 4 populations?
Definition
never-smokers, females, younger than 60, NSCLC
Term
where anatomically does SCLC usually occur in the lung
Definition
centrally, endobronchial
Term
what are 4 hormones produced by SCLC
Definition
ADH, ACTH, gastrin releasing peptide, ANP
Term
what is the typical anatomical location of SCC
Definition
central
Term
what is the typical anatomical location of adenocarcinoma
Definition
peripheral
Term
what is the definition of bronchoalveolar carcinoma
Definition
it is a subtype of adenocarcinoma that is characterized by growth along side the alveoli w/o invading it. it may appear as a mass, multicentric or diffuse
Term
what is the typical anatomical location of large cell carcinoma of the lung?
Definition
peripheral
Term
what histologically is typical of large cell carcinoma of the lung
Definition
undifferentiated
Term
what % of NSCLC are curable by surgery?
Definition
30%
Term
what are common immunohistochemical markers used in the Dx of SCC of lung
Definition
CK5,6, cytokeratin cocktail
Term
what are common immunohistochemical markers used in the Dx of adenocarcinoma of lung
Definition
CK7
TTF-1
cytokeratin cocktail
Term
what are common immunohistochemical markers used in the Dx of large cell carcinoma
Definition
cytokeratin
Term
what are common immunohistochemical markers used in the Dx of SCLC?
Definition
cytokeratin cocktail
TTF-1
CD-56
chromogranin
synaptophysin
Term
what type of cell is the precursor of adenocarcinoma of the lung
Definition
type 2 alveolar cell
Term
genes somatically altered in adenocarcinoma of lung - 3 oncogenes and 3 TSGs
Definition
EGFR, KRAS, ALK
P53, p16/14, STK11
Term
genes somatically altered in SCC of lung- 3 oncogenes and 2 TSGs
Definition
EGFR, PIK3CA, IGF-1R
p53, p63
Term
genes somatically altered in SCLC - 2 oncogenes and 3 TSGs
Definition
BCL-2, MYC
RB1, p53, FHIT
Term
where is an early somatic gene alteration detected in all lung cancer in histologicaly normal smokers lung?
Definition
chromosome 3 - TSGs
Term
what 2 TSGs show somatic alterations in 90% of SCLC
Definition
RB1, p53
Term
what is the role of screening for lung cancer with CXR or CT nowadays?
Definition
not recommended. although a large trial has shown early detection of many lung cancer, survival benefit is still not proven
Term
when nodule found on CT of lung and there is little if any doubt that its malignant, what should be the next step in mgmt?
Definition
surgery: video assisted thoracoscopy with visualization of mediastinal lymph nodes and frozen section and if malignant lobectomy
Term
when nodule found on CT of lung and there is doubt on whether its malignant or not, what should be the next step in mgmt?
Definition
risk should be assessed and lesion classified to either low risk - below 10% or moderate - 10-60%
Term
when nodule found on CT of lung and the risk of it being malignant is low, what should be the next step in mgmt?
Definition
serial CT follow-up at 3, 6, 12, 24 months
Term
when nodule found on CT of lung and the risk of it being malignant is moderate, what should be the next step in mgmt?
Definition
if nodule>1 cm - PET-CT
if air-bronchus present - bronchoscopy
if nodule is peripheral - FNA
if available Contrast CT
Term
2 characteristics of lung cancer in never-smokers
Definition
female, younger than average smoker/former
Term
what % of patients according to histologic subtypes are found post-mortum to have extra-thoracic metastatic disease?
Definition
SCLC - 95%, SCC-50%, adenocarcinoma-80%
Term
what is the MC life threatening metabolic disorder associated with malignancy?
Definition
PTHrp secretion
Term
what are 2 paraneoplastic syndromes that may cause hyponatremia in SCLC?
Definition
SIADH, ANP
Term
what is the mgmt of hyponatremia caused by SIADH in comparison to that by ANP
Definition
SIADH - fluid restriction and if no improvement Demeclocycline.
ANP - if fluid restriction doesn't help within 3-4 days, measure ANP levels, if elevated treat with supplemental sodium
Term
what is the manifestation of ectopic secretion of ACTH in SCLC in comparison to that in Cushing's syndrome from a pituitary adenoma?
Definition
SCLC - electrolyte disturbance (hypokalemia)
Cushing's - change in habitus
Term
what is the Tx of Cushing's caused by SCLC?
Definition
Tx of the underlying disease or in extreme cases bilateral adrenalectomy.
the standard medication don't help - ketoconazole, Metyrapone
Term
what is a connective tissue symptom occuring in 30% with lung cancer, usually NSCLC?
Definition
clubbing
Term
which neurologic symptoms occur exclusively with SCLC within lung cancer subtypes
Definition
Lambert Eaton, retinal blindness, encephalomyelitis
Term
which neurologic symptoms occur in all lung cancers?
Definition
peripheral neuropathies, subacute cerebellar degeneration, cortical degeneration, and polymyositis
Term
paraneoplastic encephalomyelitis in SCLC is associated with what antineuronal antibodies?
Definition
anti-Hu, anti-CRMP5, and ANNA-3
Term
2 paraneoplastic cardiovascular/hematologic symptoms in lung cancer
Definition
migratory venous thrombophlebitis (Trousseau's syndrome), nonbacterial thrombotic (marantic) endocarditis
Term
which one of the following bronchogenic methods of attaining tissue is the most sensitive for lung cancer Dx, bronchial brush, bronchial wash, bronchioloalveolar lavage, and transbronchial FNA?
Definition
transbronchial FNA
Term
how does staging of lung cancer work?
Definition
2 parts: anatomical staging and physiological staging (the capacity of the patient to undergo certain therapies)
Term
what is the cutoff of standardized uptake value suspicious for malignancy on PET-CT?
Definition
2.5
Term
what is the imaging method of choice in NSCLC?
Definition
PET-CT
Term
how is PET-CT superior to the rest of the imaging techniques in NSCLC?
Definition
demonstrating mediastinal lymph nodes and extrathoracic metastases
Term
4 conditions where PET-CT may be false negative in NSCLC?
Definition
diabetes, lesions <8 mm, slow-growing tumors, concurrent infections such as tuberculosis
Term
what is the indication to use MRI in the staging of NSCLC
Definition
superior sulcus tumor to check for brachial plexus involvement
Term
what is the imaging of choice to detect brain metastasis in NSCLC?
Definition
MRI
Term
in NSCLC what are 11 CIs to curative resection?
Definition
extrathoracic metastases, SVC syndrome, vocal cord and phrenic nerve paralysis, malignant pleural effusion, cardiac tamponade, tumor within 2 cm of the carina (potentially curable with combined chemoradiotherapy), metastasis to the contralateral lung, metastases to supraclavicular lymph nodes, contralateral mediastinal node metastases (potentially curable with combined chemoradiotherapy) and involvement of the main pulmonary artery
Term
what is the recommended method of follow up for stage 3 NSCLC, concerning the fact that distant mets are highly likely and restaging may be nessecary?
Definition
beyond PET-CT, use regular brain CT/MRI
Term
what is the recommended staging regime for SCLC?
Definition
1. CT of chest and abdomen (mets to the liver and adrenals likely)
2. brain MRI - 10% positive in asymptomatic patients
3. if indicated - bone scan
Term
in NSCLC, what is T1a
Definition
tumor<2cm
Term
in NSCLC, what is T1b
Definition
tumor 2-3 cm
Term
in NSCLC what is T1
Definition
Tumor<3 cm diameter, surrounded by lung or visceral pleura, without invasion more proximal than lobar bronchus
Term
in NSCLC what is T2
Definition
Tumor >3 cm but 7 cm with any of the following:
Involves main bronchus, 2 cm distal to carina
Invades visceral pleura
Associated with atelectasis or obstructive pneumonitis extending to hilar region but not involving the entire lung
Term
in NSCLC what is T2a
Definition
T 3-5 cm
Term
in NSCLC what is T2b
Definition
T 5-7 cm
Term
in NSCLC what is T3
Definition
Tumor >7 cm or directly invades any of the following: chest wall (including superior sulcus tumors), phrenic nerve, mediastinal pleura, parietal pericardium

Tumor <2 cm distal to carina but without involvement of carina

Tumor with associated atelectasis or obstructive pneumonitis of entire lung

Separate tumor nodule(s) in same lobe
Term
in NSCLC what is T4
Definition
Tumor of any size that invades any of the following: mediastinum, heart or great vessels, trachea, recurrent laryngeal nerve, esophagus, vertebral body, carina

Separate tumor nodule(s) in a different ipsilateral lobe
Term
in NSCLC what is N1
Definition
ipsilateral peribronchial and/or hilar lymph node(s) and intrapulmonary node(s),
Term
in NSCLC what is N2
Definition
ipsilateral mediastinal and/or subcarinal lymph node(s)
Term
in NSCLC what is N3
Definition
contralateral mediastinal, hilar, ipsilateral or contralateral scalene or supraclavicular lymph node(s
Term
in NSCLC what is M1a
Definition
Separate tumor nodules in a contralateral lobe

Tumor with pleural nodules or malignant pleural or pericardial effusion
Term
in NSCLC what is M1b
Definition
distant metastasis
Term
in NSCLC what is stage IA
Definition
T1a-T1bN0M0
Term
in NSCLC what is stage IB
Definition
T2aN0M0
Term
in NSCLC what is stage IIA
Definition
T1a-T2aN1M0 or T2bN0M0
Term
in NSCLC what is stage IIB
Definition
T2bN1M0 or T3N0M0
Term
in NSCLC what is stage IIIA
Definition
T1a-T3N2M0 or T3N1M0 or T4N0-1M0
Term
in NSCLC what is stage IIIB
Definition
T4N2M0 or T1a-T4N3M0
Term
in NSCLC what is stage IV
Definition
Any T Any N M1a or M1b
Term
what is the staging system for SCLC?
Definition
limited disease=anything that can be encompassed in one radiation port
extensive disease=cannot be contained in one radiation port
Term
3 conditions that are present outside of the hemithorax and still may be considered as limited disease in SCLC?
Definition
contralateral supraclavicular nodes, recurrent laryngeal nerve involvement, and SVC obstruction
Term
3 conditions that are present outside of the hemithorax and cannot be encompassed in one radiation port, and therefore indicate extensive disease in SCLC?
Definition
Cardiac tamponade, malignant pleural effusion, and bilateral pulmonary parenchymal involvement
Term
how many patients are diagnosed with extensive disease at presentation in SCLC?
Definition
70%
Term
which patients with NSCLC can tolerate a pneumonectomy?
Definition
FEV1>2 L or > 80% of predicted
Term
which patients with NSCLC can tolerate a lobectomy?
Definition
FEV1>1.5 L
Term
in assessing the ability of a patient with lung cancer to undergo surgery, in patients with borderline lung function but a resectable tumor, what is the next step?
Definition
cardiopulmonary exercise testing. A Vo2max <15 mL/(kg·min) predicts for a higher risk of postoperative complications
Term
after assessing the ability of a patient with lung cancer to undergo surgery, what do you do with patients deemed unable to tolerate lobectomy or pneumonectomy from a pulmonary functional standpoint?
Definition
limited resections, such as wedge or anatomic segmental resection - more recurrences, lower survival
Term
what cardiopulmonary findings in the assessment of the ability of the patient with lung cancer to undergo surgery, are considered absolute CIs to surgery?
Definition
myocardial infarction within the past 3 months (associated with 20% reinfarction), uncontrolled arrhythmias, FEV1 < 1 L, PCO2>45, DLCO <40%, and severe pulmonary hypertension
Term
what cardiopulmonary finding in the assessment of the ability of the patient with lung cancer to undergo surgery, is considered a relative CI to surgery?
Definition
infarction in the past 6 months
Term
in lung cancer, what is Tx?
Definition
malignant cells are identified in a sputum or bronchial washing specimen but the chest imaging appears normal
Term
in lung cancer, what do you do with patients who have Tx stage tumor?
Definition
must localize the lesion - 90% will be identified by examination of the bronchial tree with a fiberoptic bronchoscope under general anesthesia and collection of a series of differential brushings and biopsies
Term
after a tumor was localized in Tx stage of lung cancer and resected, what is the next step?
Definition
close follow up b/c in 5% of cases (per year) there will be a 2nd primary in the lung
Term
in the evaluation of a solitary pulmonary nodule, when would be PET-CT be useful?
Definition
lesion > 7 mm
Term
what are the 5 variables taken into account in the risk stratification of patients with a newfound solitary pulmonary nodule? (values in brackets indicate intermediate risk)
Definition
diameter (1.5-2.2 cm), age (45-60), smoking cessation status(quit less than 7 yrs ago), smoking status (less than 20 cigarettes/day), nodule margins (low-smooth, int.-scalloped, high-radiata, spiculated)
Term
what are the only two radiographic criteria thought to predict the benign nature of a solitary pulmonary nodule?
Definition
lack of growth over a period >2 years and certain characteristic patterns of calcification: a dense center, multiple punctate foci, and "bull's-eye" (granuloma) and "popcorn ball" (hamartoma)
Term
what are signs suggesting a malignancy in the evaluation of a solitary pulmonary nodule?
Definition
large lesion, lack of or asymmetric calcification, chest symptoms, associated atelectasis, pneumonitis, or growth of the lesion revealed by comparison with an old x-ray or CT scan or a positive PET scan
Term
what is the 5 yr survival for stage 1 or 2 NSCLC patients undergoing surgery?
Definition
stage 1 - 60-80%
stage 2 - 40-50%
Term
what is the 5 yr survival for stage 1 or 2 NSCLC patients undergoing surgery?
Definition
stage 1 - 60-80%
stage 2 - 40-50%
Term
what is the recommendation on lymph node sampling/dissection in the process of NSCLC resection?
Definition
complete mediastinal node dissection and hilar node sampling - higher survival rates than just sampling
Term
what is the role of adjuvant radiotherapy for NSCLC stages 1 and 2?
Definition
none, radical radiotherapy may be used in patients who are refusing pulmonary resection or are not physically fit to undergo surgery
Term
what is the 5 yr survival associated with radical radiotherapy for NSCLC stage 1 or 2?
Definition
13-39%
Term
apart from radical radiotherapy for the Tx of NSCLC, what are 2 other radiation therapy methods
Definition
cryoablation (T>3 cm) , stereotactic (T < 5 cm)
Term
what is the recommended follow up regimen for post resection NSCLC stages 1 and 2?
Definition
chest CT with contrast every 6 months for the first 2 years after surgery, followed by yearly CT scans of the chest without contrast thereafter
Term
what is the role of adjuvant chemotherapy for patients with NSCLC?
Definition
stages IB, II and III (N0 or N1 disease only)
Term
what is the recommended regimen of adjuvant chemo in NSCLC
Definition
a 2 drug combination containing cisplatin
Term
in what 3 condition should a patient indicated for adjuvant chemo Tx receive Carboplatin instead of Cisplatin?
Definition
reduced renal function, presence of neuropathy, or hearing impairment
Term
what is the Tx of N2 and N3 NSCLC?
Definition
chemoradiation (no surgery with an exception to N2 disease which shows greater survival performing lobectomy instead of pneumonectomy)
Term
what is the mgmt of patients with NSCLC evaluated clinically to have an N1 or N0 disease and found on surgery/pathology to have an N2 disease? how many patients are like that?
Definition
if it is single-station and possible to resect all the mediastinal lymph nodes - continue as planned
if multinodular, bulky, extranodular or not possible to resect all mediastinal lymph nodes - abort surgery, give chemoradiation
Term
what stage NSCLC are recommended chemoradiation?
Definition
IIIB and IIIA bulky disease
Term
what is the definition of bulky disease in stage IIIA NSCLC?
Definition
lymph nodes >2 cm, which have extranodal involvement or multistation disease
Term
in chemoradiation (non surgical) Tx of NSCLC, which regimen entails higher rates of survival and side effects, sequential or concurrent
Definition
concurrent is associated with better survival, but more side effects: fatigue, esophagitis, and neutropenia
Term
what are the symptoms of pancoast tumor in NSCLC?
Definition
Horner's syndrome, shoulder and/or arm pain, and weakness and atrophy of the muscles of the hand
Term
what is the Tx of patients with N0/N1 disease and pancoast tumor
Definition
neoadjuvant therapy
Term
what % of NSCLC patients present with advanced disease (stage IIIB with a pleural effusion or stage IV) at the time of diagnosis
Definition
66%
Term
which NSCLC patients have a better Px, those that present with metastatic disease or those that recur with metastatic disease
Definition
recurrence
Term
what is the Tx of advanced NSCLC and the prognosis?
Definition
supporative, 5 yr survival is 10% median survival 4-5 months
Term
patients with advanced NSCLC show a survival benefit with chemotherapy, how many cycles should they undergo
Definition
maximum 6
Term
what is the median survival in advanced NSCLC treated with chemo?
Definition
8-10 months
Term
in NSCLC what is the preferable regimen of chemotherapy according to tumor histology?
Definition
SCC - cisplatin and pemetrexed
non-SCC - cisplatin and Gemcitabine
Term
what is the role of Bevacizumab(Avastin) in the Tx of NSCLC
Definition
advanced disease that is non-squamous (higher risk of bleeding) in conjunction with the specific regimen of Carboplatin/paclitaxel - in the USA and not in europe
Term
3 Tx options for second-line therapy of NSCLC in the United States
Definition
docetaxel, pemetrexed, and erlotinib (Tarceva)
Term
what is the major side effect of docetaxel in comparison to the rest of the 2nd line chemo options in NSCLC?
Definition
neutropenic fever
Term
what side effect of erlotinib (Tarceva) occurs more commonly than the other 2 options for 2nd line chemo options in NSCLC?
Definition
diarrhea and rash
Term
4 Clinical features that have been shown to correlate with responsiveness to EGFR TKI treatment
Definition
female sex, never smoking status, adenocarcinoma histology, and Asian ethnicity
Term
which EGFR antibody showed improved survival in caucasians and not asians in advanced NSCLC in combination with Cisplatin/paclitaxel?
Definition
Cituximab
Term
in which malignancy does a KRAS mutation predict responsiveness to the EGFR inhibitor Cituximab? in which it doesn't
Definition
colon cancer. doesn't predict in NSCLC
Term
which Txs have been proven to show improved survival in a subset of asian patients with advanced NSCLC in comparison to the standard 1st line chemo of Carboplatin based doublet therapy?
Definition
gefitinib and erlotinib
Term
what is the only therapy approved by the U.S. FDA following platinum-based chemotherapy in patients with advanced NSCLC
Definition
pemetrexed (folate antimetabolite)
Term
what is the definition of maintanance Tx for advanced NSCLC
Definition
single agent therapy for patients who are done with their regimen of chemotherapy and are progression free either with complete or partial response
Term
which agent has been shown to improve survival as maintenance Tx in advanced NSCLC following platinum-based therapy?
Definition
Erlotinib (Tarceva)
Term
what are 2 first line options for SCLC-LD?
Definition
1. platinum based + etoposide
2. Cyclophosphomide, Vincristine, doxorubicin
Term
what is the only 2nd line treatment approved for SCLC-LD?
Definition
Topotecan
Term
whats considered a platinum resistant disease in SCLC-LD?
Definition
relapse within 3 months
Term
what is the 5 yr survival of SCLC-LD and ED? what are the median survivals?
Definition
6-12%, 2%, 12-20 months, 7 to 11 months
Term
what are the response rates to chemotherapy in SCLC?
Definition
80% (high!)
Term
what is the full Tx of SCLC?
Definition
chemoradiation with platinum based drug and etoposide
Term
what is an adjuvant therapy to patients with SCLC who have responded to initial Tx? and what effect that it have on survival?
Definition
prophylactic cranial irradiation
5.4% survival benefit in LD with complete remission
Term
what % of patients with lung cancer an EGFR mutation who respond to treatment with an EGFR TKI inhibitor (gefitinib and erlotinib) develop resistance?
Definition
all patients
Term
what is the mechanism of resistance to EGFR TKI inhibitors in lung cancer?
Definition
developing a mutation in a different TKR
Term
in lung cancer, what is a TKR that mutates in the development of EGFR TKI inhibitors resistance in 20% of cases?
Definition
MET
Term
4 Clinical characteristics associated with EML4-ALK–positive lung cancer
Definition
younger age at diagnosis, minimal smoking history, male sex, and adenocarcinoma histology with signet-ring features
Term
which biomarker predicts sensitivity to platinum chemotherapy in lung cancer?
Definition
low levels of ERCC1 - excision repair cross-complementation group 1
Term
in NSCLC, which biomarker predicts sensitivity to Gemcitabine therapy?
Definition
low RRM1 - Ribonucleotide reductase M1
Term
pemetrexed (an agent commonly employed as second-line treatment in patients with nonsquamous NSCLC) resistance can be predicted by overexpression of what biomarker?
Definition
Thymidylate synthase
Term
why is pemetrexed more effective in adenocarcinoma vs. SCC/SCLC?
Definition
the latter have higher levels of Thymydilate synthase
Term
what % of lung tumors are benign
Definition
50%
Term
what are the 2 types of benign lung tumors?
Definition
hamartomas and bronchial adenoma
Term
4 characteristics of lung hamartomas?
Definition
M>F, median age 60, peripheral, popcorn pattern on CXR
Term
what 3 type of bronchial adenomas are there?
Definition
carcinoid 80%, adenocystic tumors, or mucoepidermoid tumors (2–3%).
Term
characteristics of bronchial adenoma
Definition
central (can cause pneumonia), mean age 45, can metastasize, high vascular (bleed with Bx), 5 yr survival 95%, Tx is surgical resection
Term
2 of the most significant risk factors for head and neck cancer
Definition
alcohol and tobacco
Term
8 risk factors for head and neck cancer
Definition
alcohol, tobacco, marijuana, occupational diseases (nickel refining, textile, woodworking), low consumption of fruits and vegetables, EBV, HPV, salted fish
Term
histopathologically, into what 2 classes would you divide nasopharyngeal carcinomas? which is more common?
Definition
differentiated SCC vs. nonkeratinizing and undifferentiated carcinoma (lymphoepithelioma) that contains infiltrating lymphocytes, is more common and is associated with EBV
Term
most parotid tumors are benign or malignant?
Definition
benign
Term
in which malignancy are patients at a greater risk of dying from a second malignancy than a recurrence of the primary disease?
Definition
head and neck cancer
Term
a second primary in head and neck cancer would be caused from the same risk factors that led to the first one or from radiation therapy given for Tx of the first one?
Definition
same risk factors - carcinogens
Term
4 clinical manifestations of carcinoma of the nasopharynx
Definition
serous otitis media due to obstruction of the eustachian tube, nasal obstruction, epistaxis, neuropathies of the cranial nerves
Term
2 of the presenting symptoms of oral carcinoma?
Definition
pain and nonhealing ulcers
Term
in a primary of unknown origin found in cervicl lymph node and showing SCC histology, what is the likely origin? what is the workup?
Definition
head and neck. Bx of base of tongue, nasopharynx, tonsils, pyriform sinus
Term
what is the significance of leukoplakia or erythroplakia?
Definition
are premalignant and require Bx
Term
what is the workup of a patient who has been diagnosed with a head and neck cancer
Definition
1. CT of head to asses extent of disease
2. if lymph node involvement - CXR, bone scan, PET to assess for metastases
3. thorough endoscopic examination with multiple Bxs
Term
what is considered localized disease in head and neck cancer?
Definition
T1 or T2, without lymph node involvement or metastases
Term
what are 2 Tx options for localized head and neck cancer? and which is adequate in what circumstances?
Definition
radiation therapy - e.g. laryngeal cancer to preserve voice function
surgery - e.g. small lesions in the oral cavity to avoid the long-term complications of radiation, such as xerostomia and dental decay
Term
what is the 5 yr survival of limited head and neck cancer?
Definition
60-90%
Term
what % of head and neck cancer present with metastases?
Definition
10%
Term
what is locally or regionally advanced head and neck cancer?
Definition
disease with a large primary tumor and/or lymph node metastases
Term
what is the Tx of locally or regional advanced head and neck cancer?
Definition
intermediate stage: surgery (if resectable) and post operative chemoradiation
otherwise: neoadjuvant chemoradiation and surgery
Term
what % of patients present with locally or regionally advanced head and neck cancer?
Definition
over 50%
Term
what is the intent in the Tx of locally or regionally advanced head and neck cancer?
Definition
curative
Term
in advanced head and neck cancers, what is the goal in induction chemotherapy and sequential radiotherapy instead of concurrent neoadjuvant chemoradiation?
Definition
it leads in some cases to complete clinical remission and therefore is organ sparing
Term
which head and neck cancers show increased survival when treated with a modality which includes cisplatin and radiation therapy?
Definition
HPV associated and nasopharyngeal carcinoma
Term
what is a major toxicity associated with concomitant chemoradiation for the treatment of advanced head and neck cancers?
Definition
mucositis
Term
which monoclonal antibody is added to the chemoradiation regimen of advanced head and neck cancer Tx?
Definition
Cetuximab
Term
what is the most common treatment modality of metastatic head and neck cancer? median survival? response rates? duration of response?
Definition
chemotherapy, 6-8 months, 30-50% response rate, 3 months duration of response
Term
in metastatic head and neck cancer what are some agents used in chemotherapy and in what way?
Definition
mono/double therapy: MTX, 5FU, paclitaxel, docetaxel, cisplatin
Term
which monoclonal antibody to EGFR may be added to chemotherapy of metastatic head and neck cancer? and what is its contribution to Tx?
Definition
Cetuximab, improves median survival
Term
what is a side effect of radiation therapy seen in 50% of patients?
Definition
hypothyroidism
Term
how much time after recovery is it plausible to present with salivary gland metastatses?
Definition
20 yrs
Term
risk factors for melanoma (2 mutations)
Definition
nevi, family history, uv exposure, light skin/eyes/hair, freckling, inability to tan, CDKN2A mutation, MC1R variants
Term
what is the lifetime risk of melanoma with giant nevus (>20cm)?
Definition
6%
Term
what is the single greatest risk factor for melanoma?
Definition
personal history of melanoma
Term
what % of melanomas are familial? what are survival rates in comparison to sporadic melanoma?
Definition
10%, better survival
Term
what is another malignancy seen among melanoma-prone families with CDKN2A mutations?
Definition
pancreatic cancer
Term
what is the role of the CDKN2A gene in the healthy w/o melanoma?
Definition
encodes 2 proteins, p16 and ARF(p14), which inactivate a protein that itself inactivates p53 and Rb
Term
what are 5 types of melanoma? what is distinct to 3 of those types?
Definition
lentigo maligna melanoma, superficial spreading melanoma, acral lentiginous - have a superficial (radial) growth phase that allows early detection and excision before vertical growth and invasion.
nodular melanoma, desmoplastic melanoma - only vertical (invasive) growth
Term
which of the melanoma types grow on your back?
Definition
superficial spreading melanoma
Term
which melanoma type grows on the soles of your feet?
Definition
acral lentiginous melanoma
Term
what is the most common type of melanoma observed in caucasian population?
Definition
superficial spreading melanoma
Term
which melanoma invaded neural tissue?
Definition
desmoplastic melanoma
Term
what is the chararcteristic of melanomas in which a somatic in BRAF occurs?
Definition
they present in areas of intermittent and not chronic sun exposure
Term
5 major somatic mutations (not germ line mutation) that occur in melanoma
Definition
N-RAS, B-RAF, PI3K, PTEN, AKT
Term
what nevus size suggests malignancy?
Definition
over 6 mm (pencil eraser)
Term
which way should Bx from a suspicious nevus be taken?
Definition
deepest part and most superficial part, shaving or cauterization are not acceptable
Term
what is the least of information that should be included in a Bx report
Definition
Breslow thickness (from deepest to most superficial), mitoses per square millimeter for lesions < 1mm, presence or absence of ulceration, and peripheral and deep margin status
Term
which test can be made to distinguish melanomas from benign nevi in cases with challenging histology?
Definition
FISH
Term
what is The best predictor of metastatic risk in melanoma?
Definition
breslow thickness
Term
5 important prognostic factors for melanoma (also incorporated in the staging system)
Definition
Breslow thickness, ulceration, nodal involvement, LDH, distant metastases
Term
what is a common infection caused by radical mastectomy, the loss of lymph node drainage?
Definition
cellulitis
Term
what is the prophylactic treatment of choice in a patient with malignancy who has undergone splenectomy?
Definition
amoxicillin/clavulonate to avert from overwhelming sepsis with H.Flu, N.meningitides, Strep.Pneumonia
Term
which 3 malignancies are associated with dysfunction of phagocytosis d/t lack of neutrophils? what organisms infect?
Definition
hairy cell leukemia, ALL, AML
Staphylococci, streptococci, enteric organisms, fungi
Term
what 2 malignancies cause dysfunction of the humoral system? what are 2 manifestations
Definition
CLL, MM
pneumonia, sinusitis - encapsulated organisms
Term
which 3 malignancies cause dysfunction of the cellular immune system? which organism infect?
Definition
Hodgkin's disease, leukemia, T cell lymphoma
intracellular organisms - TB, listeria, fungi, HSV etc.
Term
in what 2 malignancies should patients receive Abx prophylaxis throughout chemotherapy for pneumocystis?
Definition
hodgkin's and ALL
Term
patients with conditions/malignancies associated with t-cell dysfunction could have serious infections with the lack of....
Definition
fever. hodgkin's, hairy cell, glucocorticoids
Term
patients with malignancies that cause neutropenia may have what unique manifestations of which common conditions?
Definition
AML/ALL
pneumonia w/o sputum or CXR findings
cellulitis w/o purulence
Term
what are the immune def. typical of hodgkin's patients?
Definition
t cell dysfunction, splenctomized
Term
which viral vaccines are indicated for hodgkins patients and which are CIed?
Definition
HPV, influenza
CI - MMR, VZV
Term
in which 3 conditions are MMR and VZV vaccinations CIed?
Definition
intensive chemotherapy, hodgkin's disease and HSCT (MMR can be given 24 months after procedure given there's no GVHD)
Term
which disease may be reactivated with the use of Rituximab (B-cell antibody)?
Definition
TB, CMV, HBV
Term
which organism not so typical can cause which skin condition in neutropenic patients?
Definition
ecythema gangrenosum (central necrosis) in nonpressure areas caused by P.aeroginosa
Term
which 3 organisms are often associated with IV catheter infection in a patient with granulocytopenia/
Definition
JK bacillus, Acinetobacter, non-aeroginosa pseudomonas
Term
5 gram + cocci that may cause infection in the neutropenic patient/
Definition
Staphylococcus epidermidis Staphylococcus aureus
Viridans Streptococcus
Enterococcus faecalis
Streptococcus pneumoniae
Term
8 gram - bacilli which may cause infection in the neutropenic patient?
Definition
Escherichia coli
Klebsiella spp.
Pseudomonas aeruginosa
Non-aeruginosa Pseudomonas spp.a
Enterobacter spp.
Serratia spp.
Acinetobacter spp.a
Citrobacter spp.
Term
2 gram + bacilli that may cause infection in the neutropenic patient?
Definition
Diphtheroids
JK bacillusa
Term
2 Fungi that may cause infection in the neutropenic patient?
Definition
Candida spp.
Aspergillus spp.
Term
what is sweet's syndrome?
Definition
febrile neutropenic dermatosis
Term
what is the dermatologic manifestation of sweet's syndrome?
Definition
red/bluish papules that coalesce to form sharp-bordered plaques
Term
what are the typical anatomical territories of the dermatologic manifestation of sweet's syndrome?
Definition
arms, neck and face
Term
what is the Tx for sweet's syndrome?
Definition
high dose glucocorticoids (60mg prednisone) followed by tapering for 2-3 wks
Term
what is a dermatologic diagnostic challenge in immunocompromised patients d/t malignancy who are both at increased risk of HSV, use multiple drugs, and may develop GVHD?
Definition
distinguishing btwn erythema multiforme (HSV) and SJS (drugs) and the rash typical to GVHD
Term
what is an indication for immediate
IV tunneled-catheter removal in the in the patient undergoing chemotherapy?
Definition
red streak over the subcutaneous part of the line
Term
what is not an immediate indication for removal of a tunneled-IV catheter in the patient undergoing chemotherapy?
Definition
erythema over the exit port
Term
what is the Tx for IV catheter infection with blood cultures positive for coagulase negative staphylococci
Definition
1. IV catheter optimal
2. Vancomycin (alternatives - Linezolid, quinupristin/dalfopristin, and daptomycin)
Term
which organisms causing IV catheter infections are notoriously hard to treat, and therefore should always prompt immediate removal of IV catheter?
Definition
Stenotrophomonas, Pseudomonas, Burkholderia, Fungal, Bacillus, Corynebacterium, Mycobacterium, Staph. Aureus
Term
IV catheter exit site erythema in the patient receiving chemo is usually caused by which organisms and how are they treated
Definition
coagulase negative staphylococci, vancomycin
Term
which bacteria is common in mouth ulceration d/t cytotoxic chemo?
Definition
strep. viridans
Term
which drug is 1st line for the Tx of both localized (thrush) and systemic (esophagitis) infection with candida albicans? what is are 2 alternative options in case of resistance to this first drug?
Definition
fluconazole
voriconazole, echinocandins (cell wall glucan inhibitors, caspofungin)
Term
Noma (cancrum oris), a disease where bacteria invade both hard and soft oral tissues, is common in what 4 conditions? which 2 bacteria are responsible?
Definition
immunocompromised, debilitated, malnourished, low hygine
Bacteroides, Fusobacterium
Term
what is the likely diagnosis in a patient with hematologic malignancy who has recently recovered from neutropenia and has persistent fever unresponsive to antibiotics, abdominal pain and tenderness or nausea, and elevated serum levels of ALP?
Definition
hepatic candidiasis
Term
how is the diagnosis of hepatic candidiasis done?
Definition
imaging(avoid Bx b/c risk of bleeding)
bull's eye lesion (granulomatous lesion)
Term
what is a possible diagnosis in a patient who is immunosuppressed with fever and right-lower-quadrant tenderness
Definition
Typhillitis (necrotizing colitis, neutropenic colitis, necrotizing enteropathy, ileocecal syndrome, and cecitis)
Term
in which conditions do you see Typhillitis?
Definition
AML,ALL, HIV
Term
how is the diagnosis of typhillitis done?
Definition
finding of a thickened cecal wall on CT, MRI, or ultrasonography
Term
chemotherapy induced diarrhea can be caused by which organism?
Definition
C.defficile
Term
The presentation of meningitis in patients with which malignancies would be caused by which 2 organisms?
Definition
lymphoma, CLL, chemotherapy for solid malignancies and BMT
cryptococcus and listeria
Term
A predisposition to encephalitis caused by intracellular organisms similar to those encountered in patients with AIDS is seen in cancer patients receiving what Tx?
Definition
(1) high-dose cytotoxic chemotherapy (2) chemotherapy affecting T cell function (e.g., fludarabine), or (3) antibodies that eliminate T cells (e.g., anti-CD3, alemtuzumab, anti-CD52)
(4) cytokine activity (anti-tumor necrosis factor agents or interleukin 1 receptor antagonists)
Term
what are 3 possible organisms causing a brain mass in patients with cancer and prolonged neutropenia?
Definition
Aspergillus, Nocardia, or Cryptococcus
Term
what are 3 possible organisms causing a brain mass in patients with cancer and defects in cellular immunity?
Definition
EBV and Toxoplasmosis
Term
what are 6 possible organisms causing diffuse encephalitis in patients with cancer and defects in cellular immunity?
Definition
VZV, CMV, HSV, HHV-6, JC virus (PML), Listeria
Term
whats a possible organism causing diffuse encephalitis in patients with cancer and prolonged neutropenia?
Definition
PML (JC virus)
Term
what is the imaging of choice for a suspected pulmonary infection in the neutropenic patient?
Definition
high-resolution CT
Term
if detecting a pulmonary lesion on high resolution CT in the immunocompromised patient what should be the next step in the workup?
Definition
check for platelet levels and elevate them to standard levels, so the next step you could perform and effective culture with the fluid you aspirate via endobronchial lavage
Term
in the immunocompromised patient with imaging proven pulmonary lesion, what organisms should you look for in the culture of the fluid aspirated?
Definition
Mycoplasma, Chlamydia, Legionella, Nocardia, PCP, fungi
Term
in the Differential Diagnosis of Chest Infiltrates in Immunocompromised Patients, what is the infectious cause of a localized infiltrate and what are 3 non infectious causes?
Definition
bacteria (pneumonia)
local hemorrhage, embolism, tumor
Term
in the Differential Diagnosis of Chest Infiltrates in Immunocompromised Patients, what are 2 possible infectious causes of a lobular infiltrate and what is a possible noninfectious cause?
Definition
fungi, nocardia
recurrent tumor
Term
in the Differential Diagnosis of Chest Infiltrates in Immunocompromised Patients, what are possible infectious causes of a diffuse infiltrate and what are possible noninfectious causes?
Definition
Viruses (especially CMV), Chlamydia, Pneumocystis, Toxoplasma gondii, mycobacteria

Congestive heart failure, radiation pneumonitis, drug-induced lung injury, diffuse alveolar hemorrhage (described after BMT
Term
what is the "crescent sign" on chest x-ray of an immunocompromised patient?
Definition
resolving aspergillus infection or invasive aspergillus infection
a necrotic part of the lesion in its center
Term
The appearance of a discolored area in the nasal passages or on the hard palate of an immunocompromised patient should prompt a search for what?
Definition
invasive aspergillus
Term
If an immunocompromised patient has a diffuse interstitial pattern on chest x-ray, it may be reasonable, while considering invasive diagnostic procedures, to institute empirical treatment with what?
Definition
for Pneumocystis with TMP-SMX and for Chlamydia, Mycoplasma, and Legionella with a quinolone or an erythromycin derivative (e.g., azithromycin)
Term
what is the most common cause of chemotherapy-induced lung disease
Definition
Bleomycin
Term
causes of chemotherapy-induced lung disease
Definition
bleomycin, alkylating agents (such as cyclophosphamide, chlorambucil, and melphalan), nitrosoureas [carmustine (BCNU), lomustine (CCNU), and methyl-CCNU], busulfan, procarbazine, methotrexate, and hydroxyurea
Term
what is The treatment of radiation pneumonitis?
Definition
glucocorticoids
Term
what might infect the thyroid in an immunocompromised patient?
Definition
candida
Term
what might infect the adrenal in an immunocompromised patient?
Definition
CMV
Term
2 viruses that may cause hemorrhagic cystitis in the immunocompromised patient
Definition
BK virus, adenovirus
Term
patients undergoing intensive chemotherapy for any form of cancer will have defects in which components of the immune system?
Definition
granulocytes and lymphoctes dysfunction
Term
what is the length of Abx Tx in the neutropenic patient?
Definition
until at least 2 days where the neutrophil count stays above 500
Term
if patients remain febrile after resolution of neutropenia the following 3 diagnoses should be seriously considered
Definition
(1) fungal infection, (2) bacterial abscesses or undrained foci of infection, and (3) drug fever
Term
what 4 things shouldn't be missed in the PE of a patient presenting with febrile neutropenia?
Definition
perianal area, skin lesions, mucous membranes, IV catheter sites
Term
in the workup of a febrile neutropenic patient, who has begun emperical Abx Tx, but the source of infection hasn't been found and the patient's fever persists, what is the next step in mgmt?
Definition
add an antifungal agent
Term
what is the most important rule about choosing an Abx Tx for a neutropenic patient?
Definition
it should cover both gram + and - bacteria
Term
studies shows benefit for the use of prophylactic Abx in patients that are expected to undergo neutropenia, which agent?
Definition
ciprofloxacin or levofloxacin
Term
5 fungal infections that are typical of neutropenic cancer patients
Definition
Candida, Aspergillus, Fusarium, Trichosporon, Bipolaris
Term
which fungal infection very common in immunocompromised patients of other causes, isn't seen in AML patients undergoing chemo?
Definition
cryptosporidium
Term
what is the Tx of choice for fungal infection in the neutropenic cancer patient? and which fungus does it not cover?
Definition
Voriconazole, Mucor
Term
why isn't Fluconazole adequate for the Tx of a neutropenic cancer patient with a fungal infection?
Definition
it doesn't cover aspergillus or non-albicans Candida species
Term
which antiviral drug is given for the prophylaxis of a viral infection (HSV, VZV, CMV, EBV) in the neutropenic cancer patient?
Definition
Valacyclovir
Term
which 3 antiviral drugs cover HHV-6 (sixth disease in kids) in the neutropenic cancer patient?
Definition
gancyclovir, valgancyclovir, foscarnet
Term
which 2 antiherpesviridae drugs are nucleic acid analogues? the rest work in what mechanism
Definition
Famcyclovir (Pencyclovir), Cidofovir
Polymerase inhibitors - either specific for HSV (acyclovir) or broader
Term
which 3 antiherpesviridae drugs may cause bone marrow suppression?
Definition
Gancyclovir, Valgancyclovir, Cidofovir
Term
which antiherpesviridae drug may cause thrombotic microangiopathy?
Definition
Valacyclovir
Term
which antiherpesviridae drug may cause crystaluria (nephrolithiasis) at high doses?
Definition
acyclovir
Term
which 2 antiherpesviridae drugs may cause nephrotoxicity?
Definition
cidofovir, foscarnet
Term
which antiinfluenza A drug may be given prophylactically in the neutropenic cancer patient?
Definition
Amantadine
Term
how does Amantadine work?
Definition
it interferes with the uncoating of the Influenza A virus
Term
which 2 antiviral drugs are effective against influenza A and B and are Neuroaminidase inhibitors?
Definition
Oseltamivir (TamiFlu), Zanamivir
Term
which antiviral drugs are associated with side effects of fever, myalgias and bone marrow suppression?
Definition
interferons
Term
which antiviral drug is used against HCV and RSV viruses? what is a side effect of it? mechanism?
Definition
Ribavirin, anemia, purine analogue
Term
which prophylactic Tx is important in AML and ALL patients undergoing chemotherapy?
Definition
Resprim for PCP
Term
what is the role of granulocyte transfusion in the neutropenic cancer patient?
Definition
as a Tx for patient who is unresponsive to Abx Tx (not to be used prophylactically)
Term
what is the recommended use of cytokine therapy in the neutropenic cancer patient (G-CSF, GM-CSF)?
Definition
in severe prolonged neutropenias only
Term
4 possible side effects of cytokine Tx (G-CSF)?
Definition
fever, hypoxemia, pleural effusions or serositis
Term
what environmental factor is associated with in hospital infections with aspergillus?
Definition
construction
Term
what is the source of most organisms that infect and invade the neutropenic cancer patient?
Definition
his own bowel and skin flora
Term
which 2 environmental health protection methods are used in hospitals but are not clinically proven to be effective in the neutropenic cancer patient?
Definition
laminar air flow, reverse isolation - visitors and staff wear gowns and gloves
Term
although not clinically proven, which foods should the neutropenic cancer patient avoid?
Definition
fresh vegetables (P.Aeroginosa), leftovers, deli foods, and unpasteurized dairy products
Term
what are the recommendations for intravenous immunoglobulin replacement therapy in the immunocompromised cancer patient? which conditions are associated with hypogammaglobulinemia?
Definition
levels<400 for a prolonged period of time
CLL, MM, recipients of BMT
Term
all cancer patients receiving chemotherapy that includes glucocorticosteroids should be prophylactically treated with?
Definition
Resprim for PCP
Term
Therapeutic radiation is delivered in three ways
Definition
(1) teletherapy, with beams of radiation generated at a distance and aimed at the tumor within the patient; (2) brachytherapy, with encapsulated sources of radiation implanted directly into or adjacent to tumor tissues; and (3) systemic therapy, with radionuclides targeted in some fashion to a site of tumor
Term
which cells are relatively resistant to radiation therapy?
Definition
hypoxemic cells and dividing cells
Term
which are the most widely used forms of radiation energy used in therapy?
Definition
x-rays, gamma rays
Term
high voltage energy radiation results in what effect on the skin and why?
Definition
skin sparing effect d/t less scatter of radiation to adjacent tissues
Term
how does radiation therapy induce cancer cell death?
Definition
formation of free radical hydroxyl groups which create breaks in the DNA
Term
5 diseases that are curative with radiation therapy?
Definition
breast cancer, Hodgkin's disease, head and neck cancer, prostate cancer, and gynecologic cancers
Term
which radionuclides are effective in controlling bone metastasis and reducing pain?
Definition
Strontium 89 and samarium 153
Term
which Cancers can be possibly cured with "High-Dose" Chemotherapy With Stem Cell Support
Definition
Relapsed leukemias,Relapsed lymphomas - Hodgkin's and non-Hodgkin's, CML, MM
Term
5 cancers that may be cured with the combination of chemo and radiotherapy
Definition
lung, cervix, anus, breast, head and neck
Term
3 cancers that may be cured with adjuvant chemotherapy to surgery
Definition
breast, colon, sarcoma
Term
which 9 cancers are poorly responsive to chemotherapy in advanced stages?
Definition
Pancreatic carcinoma
Biliary-tract neoplasms
Thyroid carcinoma
Carcinoma of the vulva
Non-small cell lung carcinoma
Prostate carcinoma
Melanoma
Hepatocellular carcinoma
Salivary gland cancer
Term
which two adverse phenomena are typical of Procarbazine, an alkylating agent?
Definition
cheese effect (acts as MAOi), disulfiram-like effect
Term
which 10 chemotherpeutic agents (belonging to 4 families) cause alopecia?
Definition
1. alkylating agents: procarbazine, cyclophosphamide, chlorambucil
2. antitumor antibiotic - Actinomycin D, Doxorubincin, Daunorubicin
3. antitopoisomerase - etoposide, irinotecan, topotecan
4. antimitotic - paclitaxel
Term
which 13 chemotherapeutic agents (belonging to 3 families) cause CNS side effects?
Definition
1. alkylating agents: Ifosfamide, Procarbazine, Altretamine, Oxaliplatin
2. antimetabolites: Deoxycoformycin (ADA inhibitor), hydroxyurea, Cytosine arabinoside, Azacytidine, Fludarabine, MTX, 5-FU
3. antimitotic agents: Vincristine
Term
which 7 chemotherapeutic (belonging to 4 families) agents cause neuropathy?
Definition
1. alkylating agents: Ifosfamide, Cisplatin
2. anti-mitotic agents: paclitaxel (sensory), Docetaxel, Ixabepilone
3. proteosome inhibitors: Bortezomib (Velcade - relapsed MM/mantle cell lymphoma)
4. arsenic trioxide (2nd line in leukemia)
Term
which 8 chemotherapeutic agents (belonging to 2 families) cause renal toxicity
Definition
1. alkylating agents: Carmustine, Cisplatin, Carboplatin
2. antimetabolites: Deoxycoformycin (ADA inhibitor), 2-Chlorodeoxyadenosine (hairy cell leukemia), Hydroxyurea, MTX, 5-FU
Term
which 2 chemotherapeutic agents cause hand-foot syndrome
Definition
1. antimetabolites: Capcentabine (Xeloda)
2. Tyrosine-kinase inhibitors: Sorafenib (Nexavar)
Term
what increases the toxicity of 5-FU
Definition
leucovirin (same agent that rescues from MTX toxicity)
Term
which 11 chemotherapeutic agents (belonging to 5 families) cause stomatitis
Definition
1. mTor inhibitors: Everolimus, Temsirolimus
2. antimitotic agents: Docetaxel
3. antimetabolites: Cytosine arabinoside, 5-FU, MTX, Hydroxyurea
4. antitumor Abx: Doxorubicin/Daunorubicin, Actinomycin D
5. antitopoisomerase - etoposide, topotecane
Term
what are 2 adverse effects of Imatinib (Gleevec)?
Definition
Nausea, periorbital edema
Term
which 3 chemotherapeutic drugs cause Raynaud's phenomenon
Definition
1. antimitotic agents: Vinblastine
2. antitumor Abx: Bleomycin
3. alkylating agent: Cisplatin
Term
all Tyrosine kinase inhibitors cause Rash except for which 2?
Definition
Imatinib (Gleevec), Sunitinib (Sutent)
Term
which 10 chemotherapeutic agents (belonging to 5 families) cause cardiac toxicity
Definition
1. alkylating agents: cyclophosphamide
2. antitumor Abx: Doxorubicin, Idarubicin, Epirubicin, Mitoxantrone
3. histone deacetylase inhibitors: romidepsin (lymphoma)
4. antimitotic agent: Vincristine, Paclitaxel, Estramustine
5. antimetabolites: 5-FU
Term
which 6 chemotherapeutic drugs (5 families) cause hypersensitivity reactions?
Definition
1. antitumor Abx: Bleomycin
2. antitopoisomerase: Etoposide
3.antimetabolites: Asparaginase
4. antimitotic agents: Paclitaxel, Docetaxel
5. targeted toxins: Denileukin diftitox (leukemia/lymphoma - il2+diphtheria toxin)
Term
3 chemotherapeutic agents which require liver metabolism activation
Definition
alkylating agents: Cyclophosphamide, Procarbazine, Altretamine
Term
which 5 chemotherapeutic drugs (belonging to 2 families) are eliminated via hepatic clearance
Definition
1. antitopoisomerase: Etoposide
2. antimitotic agents: Vincristine, Vinblastine, Paclitaxel, Vinorelbine
Term
which chemotherapeutic agents cause cystitis, and which must be treated with Mesna?
Definition
Cyclophosphamide, Ifosphamide - must give mesna
Term
the cardiotoxicity of doxorubicin is increased when given together with what chemo drug?
Definition
Trastuzumab (Herceptin)
Term
which chemotherapeutic drug oxidises Fe+2 to create a superoxide and hydroxyl radicals?
Definition
Bleomycin
Term
which 9 chemotherapeutic agents (4 families) are vesicants? (cause fluid-filled skin blisters)
Definition
1. antimitotic agents - vincristine, vinblastine, vinorelbine
2. antitumor Abx: Mitoxantrone, Idarubicin, Actinomycin D, Doxorubicin
3. alkylating agents: Mechlorethamine (nitrogen mustard)
4. antitopoisomerase: Etoposide
Term
which chemotherapeutic agents may cause Acute Leukemia?
Definition
1. antitumor Abx: Mitoxantrone (APL)
2. antitopoisomerase: Etoposide
Term
which 3 drugs worsen renal failure (tubular) caused by MTX?
Definition
Salicylates, NSAIDs, Probenecid
Term
which antimetabolite chemotherapeutic agent should be administered with B12 and folate to decrease toxicity?
Definition
Pemetrexed
Term
which chemotherapeutic agents cause hyperglycemia
Definition
1. antimetabolites: asparaginase
Term
what medication should be given prior to Tx with the chemotherapeutic agents of the Taxane family (Paclitaxel, Docetaxel)?
Definition
corticosteroids and H1/2 blockers d/t likelihood of hypersensitivity reaction
Term
in what circumstances is Oncotype-Dx used in breast cancer decision making?
Definition
ER+, node negative disease - to check if patient might benefit from hormonal and chemo therapy
Term
6 indications for prophylaxis with G-CSF in the 1st chemotherapeutic cycle?
Definition
1. probability of febrile neutropenia is 20%
2. preexisting neutropenia or active infection
3. Age >65 years treated for lymphoma with curative intent
4. poor performance status
5. Extensive prior chemotherapy
6. dose dense regimens
Term
indication for prophylactic G-CSF in subsequent chemotherapeutic cycles (not the first cycle)
Definition
febrile neutropenia in previous cycle
Term
2 indications for the therapeutic use of G-CSF in febrile neutropenia (not used in afebrile neutropenia)
Definition
1. BMT or HSCT
2. sepsis, pneumonia, fungal infection - no proven benefit
Term
what is the indication of platelet transfusion in the leukemic patient?
Definition
fever (stressor) + PLT<20,000
Term
what is the indication of platelet transfusion in the patient with a solid tumor malignancy?
Definition
PLT<10,000
Term
what is the indication of platelet transfusion in the patient with a myeloproliferative state?
Definition
bleeding (regardless the level of platelets b/c whaplatelets are abnormal)
Term
what is the indication for the use Oprelvekin (il11)? what are the side effects?
Definition
expected thrombocytopenic state

headache, fever, malaise, syncope, cardiac arrhythmias, and fluid retention.
Term
what is the indication for the use of EPO in patients undergoing chemotherapy? side effects? target level?
Definition
therapy for >2 months on a "stable" regimen likely to require continuing transfusions
thromboembolic events, 9-10
Term
what are 3 indications of blood transfusions in the patient receiving chemotherapy?
Definition
1. Hb<8
2. Hb<9 in patient with severe cardiovascular disease
3. end organ failure
Term
what is the MC side effect of chemotherapy?
Definition
nausea
Term
which chemotherapeutic agents cause nausea in more than 90% of patients?
Definition
mechlorethamine, streptozotocin, DTIC (Dacarbazine - alkylating agent), cyclophosphamide at >1500 mg/m2, and cisplatin
Term
which chemotherapeutic agents cause nausea in 30-90% of cases?
Definition
carboplatin, cytosine arabinoside (>1 mg/m2), ifosfamide, conventional-dose cyclophosphamide, and anthracyclines
Term
4 chemotherapeutic agents that cause nausea in 10-30% of cases?
Definition
fluorouracil, taxanes, etoposide, and bortezomib
Term
what is the prophylactic Tx of the anticipated nausea from high-risk chemotherapeutic agents?
Definition
1. 5-HT3 antagonists: ondensatron etc.
2. NK1 receptor antagonists: Aprepitant
3. Dexamethasone
Term
what is the prophylactic Tx of the anticipated nausea from medium-risk chemotherapeutic agents (except cyclophosphamide and doxorubicin)?
Definition
1. 5-HT3 antagonists: ondensatron
2. Dexamehtasone
Term
what is the prophylactic Tx of the anticipated nausea from low-risk chemotherapeutic agents?
Definition
antidopaminergic - halidol, prochlorperazine, metoclopramide
benzodiazepines
Term
2 chemotherpaeutic agents that cause severe diarrhea?
Definition
1. antimetabolite: 5-FU
2. antitopoisomerase: irinotecan
Term
AKT1 is an oncogene expressed in what malignancy?
Definition
gastric
Term
AKT2 is an oncogene expressed in what malignancy?
Definition
ovarian, breast, pancreatic
Term
BRAF is an oncogene expressed in what malignancy?
Definition
melanoma, lung CRC
Term
ERBB2 (HER2) is an oncogene expressed in what malignancy?
Definition
breast, ovary, neuroblastoma, gastric
Term
what is the MC mortality associated malignancy in males and females, and 2nd MC?
Definition
Leukemia
CNS
Term
what is the MC mortality associated malignancy in males age 20-39? 2nd MC?
Definition
Leukemia
CNS
Term
what is the MC mortality associated malignancy in females age 20-39? 2nd MC?
Definition
breast
cervix
Term
what is the MC mortality associated malignancy in males age 40-59? 2nd MC?
Definition
lung
colorectal
Term
what is the MC mortality associated malignancy in females age 40-59? 2nd MC?
Definition
breast
lung
Term
3 extragonadal sites from which germ cell tumors infrequently arise
Definition
mediastinum, retroperitoneum, pineal gland
Term
germ cell tumors occur most frequently in males of what age?
Definition
20-40
Term
which syndrome is associated with mediastinal germ cell tumor?
Definition
kleinfelter's syndrome
Term
what chromosome abnormality is associated with all germ cell tumors
Definition
duplication of the short arm of chromosome 12
Term
what is a pathognomonic presentation of testicular cancer?
Definition
painless mass in the testicle
Term
what is the MC presentation of testicular cancer?
Definition
testicular swelling or discomfort
Term
what is the next step after identifying a testicular tumor in US?
Definition
radical inguinal orchiectomy
Term
staging for testicular malignancy is preformed after radical inguinal orchiectomy, what are the steps taken?
Definition
1. serum levels of hCG, AFP, LDH
2. CXR
3. CT of chest and abdomen
Term
what is the staging of testicular malignancy?
Definition
stage 1 - limited to the testis, epididymis, or spermatic cord
stage 2 - limited to retroperitoneal (regional) lymph nodes
stage 3 - outside the retroperitoneum, involving supradiaphragmatic nodal sites or viscera
Term
which 4 histologic subtypes does nonseminoma testicular cancer include
Definition
embryonal carcinoma, teratoma, choriocarcinoma, and endodermal sinus (yolk sac) tumor
Term
what does pure emryonal carcinoma (nonseminoma) secrete?
Definition
hCG or AFP or both
Term
in what stages does nonseminoma usually present
Definition
33% stage 1, 33% stage 2 and 33% stage 3
Term
in what stages does nonseminoma usually present
Definition
70% stage 1, 20% stage 2 and 10% stage 3
Term
which tumor marker is elevated in nonseminoma but not in seminoma?
Definition
AFP
Term
how should a patient diagnosed with seminoma but has elevated AFP be treated?
Definition
as nonseminoma
Term
what is the treatment of stage 1 nonseminoma after orchiectomy and declining AFP and hCG?
Definition
if T1 and no evidence of vascular or lymphatic invasion - 2 options:
1. RPLND - retroperitoneal lymph node dissection
2. observation
otherwise, only RPLND
Term
what are 2 complications of retroperitoneal lymph node dissection?
Definition
infertility
retrograde ejaculation
Term
what % of patients with non seminoma who had clinical stage 1 turned out to be pathological stage 2?
Definition
20-30%
Term
what % of patients with non seminoma clinical stage 1, T2-T4, have pathological stage 2 disease and hence should undergo RPLND?
Definition
50%
Term
what is the treatment of non seminoma stage 2 patients with limited, ipsilateral retroperitoneal adenopathy (nodes usually 3 cm in largest diameter) and normal levels of AFP and hCG?
Definition
orchiectomy and RPLND
Term
what is the Tx of patients with non seminoma stage 2, low volume retroperitoneal nodes and elevated levels of AFP and hCG?
Definition
low volume retroperitoneal disease: tumor nodes< 2 cm in diameter and <6 nodes involved
surveillance - relapse less than 33%
Term
what is the Tx of patients with non seminoma stage 2, high volume retroperitoneal nodes and elevated levels of AFP and hCG?
Definition
high volume retroperitoneal disease: >6 nodes involved, or any involved node >2 cm in largest diameter, or extranodal tumor extension
2 courses of adjuvant chemotherapy
Term
what is the regimen for stage 2 non seminoma adjuvant chemotherapy?
Definition
cisplatin + etoposide +/- bleomycin
Term
Tx of stage 1 seminoma
Definition
orchiectomy +/- radiation therapy to retroperitoneum
Term
what are the recurrence rates for stage 1 seminoma?
Definition
15%, 30% of which recur after 2 yrs and 5% recur after 5 yrs
Term
2 factors that have been associated with higher recurrence rates in stage 1 seminoma and are therefore recommended for retroperitoneal radiation?
Definition
rete testis involvement and size >4 cm
Term
what is the Tx for stage 2A (nodes<2cm) and 2B (nodes 2-5 cm non-bulky) seminoma
Definition
orchiectomy +radiation therapy to retroperitoneum
Term
what is the Tx for stage 2B and 2C (nodes>5cm, bulky) seminoma
Definition
orchiectomy + chemotherapy
Term
what are the relapse free survival rate for patients with stage 2 seminoma with retroperitoneal nodes<5 cm?
Definition
90%
Term
what is the Tx for advanced germ cell tumors, stage 2C and stage 3 (either seminoma or non-seminoma) and the rates of complete remission?
Definition
1. orchiectomy +chemotherapy alone - complete response (decreased AFP/hCG and no evidence of disease on imaging for > 1 month) - 60%
2. orchiectomy + chemo + surgery to remove residual disease
Term
in the risk stratification of advanced germ cell tumors for seminoma, what is considered good and intermediate risk?
Definition
good - absence of nonpulmonary visceral metastases
intermediate - presence of nonpulmonary visceral metastases
Term
in the risk stratification of advanced germ cell tumors for non-seminoma, what defines a good risk?
Definition
Gonadal or retroperitoneal primary site
Absent nonpulmonary visceral metastases
AFP <1000 ng/mL
Beta-hCG <5000 mIU/mL
LDH <1.5 x upper limit of normal (ULN)
Term
in the risk stratification of advanced germ cell tumors for non-seminoma, what defines an intermediate risk?
Definition
Gonadal or retroperitoneal primary site
Absent nonpulmonary visceral metastases
AFP 1000–10,000 ng/mL
Beta-hCG 5000–50,000 mIU/mL
LDH 1.5–10 x ULN
Term
in the risk stratification of advanced germ cell tumors for non-seminoma, what defines a poor risk?
Definition
Mediastinal primary site
Presence of nonpulmonary visceral metastases
AFP 10,000 ng/mL
Beta-hCG>50,000 mIU/mL
LDH > 10 x ULN
Term
which type uses markers in its risk stratification and staging, seminoma or non-seminoma?
Definition
non-seminoma
Term
what % of patients with good, intermediate and poor risk stratification for germ cell tumors achieve durable complete responses?
Definition
90%, 75%, 45%
Term
what is salvage chemotherapy (2nd and 3rd lines) in advanced germ cell tumor?
Definition
2nd line: vinblastine, ifosfamide, and cisplatin
3rd line: high-dose carboplatin (1500 mg/m2) plus etoposide (1200 mg/m2), with or without cyclophosphamide, with peripheral blood stem cell support
Term
mediastinal nonseminoma is associated with what 3 conditions
Definition
AML, MDS, essential thrombocytosis
Term
nonseminoma can preform malignant transformation into which 2 diseases:
Definition
rhabdomysarcoma or adenocarcinoma
Term
risk factors for ovarian cancer
Definition
nulliparity, use of talc agents applied to the perineum, obesity, and probably hormone replacement therapy
Term
protective factors for ovarian cancer
Definition
oral contraceptives, multiparity, and breast-feeding
Term
what % of females with ovarian cancer present with stage 3c disease - tumors with bulky intraperitoneal disease?
Definition
70%
Term
what are the stages of ovarian carcinoma?
Definition
stage I tumors confined to the ovary, stage II malignancies confined to the pelvis, and stage III confined to the peritoneal cavity
Term
what is the workup and Tx of a female with an ovarian lesion which turns out to be malignant primary?
Definition
first, make sure its malignant and primary by performing unilateral salpingo-oopherectomy. if primary cancer of the ovary - procede to hysterectomy, removal of the remaining tube and ovary, omentectomy, and pelvic node sampling along with some random biopsies of the peritoneal cavity
Term
what is the 2 step mgmt of bulky intrabdominal ovarian cancer
Definition
1. maximal tumor cytoreduction is attempted even if it involves partial bowel resection and splenectomy b/c it is associated with better survival than visible disease
2. chemo - platine + texane
Term
what is the median survival of patients with and w/o gross residual disease?
Definition
17 months vs. 39 months
Term
what % of patients with advanced stage ovarian cancer who underwent surgery + chemo, respond to therapy, go into complete remission relapse after complete remission?
Definition
70%, 40-50%, 50% relapse from complete remission
Term
what are the chemotherapeutic agents used in relapse of ovarian cancer?
Definition
gemcitabine, topotecan, liposomal doxorubicin, and bevacizumab
Term
what % of ovarian cancer are HER2+?
Definition
20%
Term
prognosis in ovarian cancer is influenced by what 2 parameters of disease?
Definition
stage and grade
Term
what are the risk factors for soft tissue sarcoma
Definition
radiation, immunosuppression, germ line mutations (p53 li fraumeni, Neufibromatosis, RB1)
Term
translocation invovled in 90% of sarcomas
Definition
t(18:X)
Term
what is the substance secreted by some sarcomas? and its effects
Definition
IGF2 - growth, metastasis, hypoglycemia
Term
which sarcoma doesn't metastasize?
Definition
well-differentiated liposarcoma
Term
what is the rate of lymph node involvement in sarcoma? which 5 diseases are exeptional
Definition
5%
synovial and epithelioid sarcomas, clear-cell sarcoma (melanoma of the soft parts), angiosarcoma, and rhabdomyosarcoma - 17%
Term
what is the MC site of metastasis in sarcoma? what are 3 exceptional diseases
Definition
lungs
GIST - liver
myoxoid liposarcoma - adipose tissue
clear cell sarcoma - bones
Term
what is the imaging of choice for Dx in sarcoma
Definition
depends on the location of tumor:
extremities or head and neck - x-ray
abdomen/chest/retroperitoneum - CT
Term
what is stage 1 sarcoma?
Definition
G1/2,T1-2a,N0,M0
well differentiated-moderately differentiated, T1<5 cm, T2>5cm, a - superficial fascial involvement
Term
what is stage 2 sarcoma?
Definition
G1/2,T2b,N0,M0, G3/4,T1-T2a,N0,M0
poorly differentiated, undifferentiated
Term
what is stage 3 sarcoma?
Definition
G3/4,T2b,N0,M0
poorly differentiated, undifferentiated, b-deep fascial involvement
Term
what is the Tx of stage 1 soft tissue sarcoma?
Definition
surgery
Term
what is the Tx of stage 2 soft tissue sarcoma?
Definition
surgery + adjuvant radiation therapy
Term
what the Tx of stage 3 soft tissue sarcoma?
Definition
surgery + adjuvant chemo: doxorubicin + ifosfamide + growth factors in high risk (T2, high grade, local recurrence)
Term
what is the Tx of advanced (metastatic) soft tissue sarcoma?
Definition
1. surgery
2. chemo - 1st line: doxorubicin + ifosphamide, 2nd line: Gemcitabine +/- Docetaxel
3. resection of metastases
Term
what % of patients with advanced soft tissue sarcoma achieve complete remission? out of those, what % achieve long term survival?
Definition
40%, 20%
Term
which benign bone lesions can turn malignant?
Definition
enchondromas, osteochondromas -> chondrosarcomas
fibrous dysplasia, bone infarcts, and Paget's disease --> osteosarcoma
Term
the stages of bone sarcomas?
Definition
stage 1 - low grade
stage 2 - high grade
stage 3 - discontinous lesions in the primary site
stage 4 - lymph node/metastases
Term
what are the MC sites of osteosarcomas?
Definition
distal femur, proximal tibia, and proximal humerus
Term
what is the incidence of osteosarcomas amongst bone sarcomas?
Definition
45%
Term
osteosarcoma has a predilection to which part of the bone?
Definition
metaphyses of long bones
Term
what is a radiographic sign of osteosarcoma?
Definition
codman's triangle - missing bone, raised periosteum, formation of new bone on the margin of the tumor
Term
what is the Tx of osteosarcoma?
Definition
Preoperative chemotherapy followed by limb-sparing surgery (which can be accomplished in >80% of patients) followed by postoperative chemotherapy
Term
which chemotherapeutic agents are used in osteosarcoma?
Definition
doxorubicin, ifosfamide, cisplatin, and high-dose methotrexate with leucovorin rescue
Term
what is the long term survival % in extremity osteosarcoma?
Definition
60-80%
Term
what is the role for radiotherapy in osteosarcoma?
Definition
none - radioresistant
Term
chondrosarcomas have a predilection for which bones?
Definition
flat bones- shoulder/pelvic girdle
Term
what is the Tx of chodrosarcoma?
Definition
surgery, chemoresistant except 2 subtypes - dedifferentiated and mesenchymal chondrosarcoma
Term
Ewing's sarcoma has a predilection for which part of the bone
Definition
diaphysis and flat bones
Term
what is the radiographic sign of Ewing's sarcoma and how does it correlate with its histology?
Definition
"onion-peel" periosteum which is d/t histologically monotoneous small blue cells resembling the appearance of other malignancies such as: small cell carcinoma, lymphoma, embryonal rhabdomyosarcoma
Term
what is a cell surface marker seen in Ewing sarcoma? derived from which gene? what does Ewing's sarcoma stain for?
Definition
p30/32, mic-2, periodic-acid-schiff (glycogen)
Term
Ewing sarcoma is a member of a family with 3 other sarcoma tumors, what is it called and what are the 3 tumors?
Definition
PNET - peripheral neuroepithelioma, Askin's tumor (chest wall), and esthesioneuroblastoma
Term
what is the classic translocation in Ewing sarcoma and other PNETs?
Definition
t(11:22)
Term
what is the Tx of Ewing sarcoma/
Definition
usually start with chemo: Doxorubicin, cyclophosphamide or ifosfamide, etoposide, vincristine, and dactinomycin
local resection and radiation therapy
Term
what is the 5 yrs survival of ewing sarcoma that presents distally to the elbow or mid calf?
Definition
80%
Term
what is the intent of Tx for 1st presentation metastatic ewing's sarcoma?
Definition
curative
Term
which 8 malignancies metastasize to bone?
Definition
prostate, breast, lung, kidney, bladder, thyroid, lymphomas and sarcomas
Term
in descending order of frequency, the sites most often involved in bone metastasis
Definition
vertebrae, proximal femur, pelvis, ribs, sternum, proximal humerus, and skull
Term
what serum marker is elevated in osteoblastic metastases?
Definition
ALP
Term
how would you be able to distinguish btwn osteoporosis and metastases on imaging?
Definition
in metastases the cortical bone is destroyed
Term
in a carcinoma of unknown primary that is located in the midline and is poorly or undifferentiated what markers should be taken?
Definition
hCG and AFP for germ cell tumors
Term
what is the workup for every patient who presents with carcinoma of unknown primary?
Definition
1. FNA/core Bx - in 60% of cases adenocarcinoma, 5% are SCC and 35% are poorly differentiated, hematoxylin & eosin stain, immunohistochemistry (CK7, CK20)
2. CXR, CT/MRI - abdomen, pelvis, chest
Term
what is the extra workup of a patient with a carcinoma of an unknown primary, which is SCC on pathology and presents as cervical lymphadenopathy?
Definition
1. indirect and direct laryngoscopy, bronchoscopy, and upper endoscopy
2.PET: guide the biopsy; determine the extent of disease; facilitate the appropriate treatment, including planning radiation fields; and help with disease surveillance
Term
what is the major indication for PET in carcinoma of an unknown primary? what is a general minor one?
Definition
cervical adenopathy
in managing a solitary tumor to help plan surgery and see if the field need to be widened
Term
what is the indication to preform endoscopy in the workup of a patient with carcinoma of and unknown primary?
Definition
symptoms/signs suggestive of a GI primary
Term
what are 2 options for CK7-, CK20+ in carcinoma of an unknown primary?
Definition
CRC, merkell cell
Term
what are 2 options for CK7+, CK20+ in carcinoma of an unknown primary?
Definition
Ovarian, Pancreatic/cholangiocarcinoma
Term
in a female with carcinoma of an unknown primary, adenocarcinoma on pathology, what are the 3 extra steps taken in the workup?
Definition
mammogram/US
if negative MRI
if negative still, chemo+/-radiation
Term
what is the extra workup of a male with bone metastasis of an unknown primary, adenocarcinoma on pathology?
Definition
PSA
if not elevated treat with chemo or radiotherapy
Term
what is the extra workup of a solitary site of metastasis of an unknown primary with adenocarcinoma pathology?
Definition
if resectable resect and treat with CRT if not resectable - CRT
Term
what is the extra workup for peritoneal metastasis of unknown primary with adenocarcinoma pathology?
Definition
if primary peritoneal - treat for ovarian cancer
if not primary peritoneal - perform GI workup and treat with chemotherapy
Term
what is the treatment of disseminated carcinoma of an unknown primary, 2 or more sites with adenocarcinoma pathology?
Definition
chemotherapy
Term
Gross cystic disease fibrous protein-15 is a immunohistochemical marker indicative of what primary?
Definition
breast cancer
Term
Thyroid transcription factor 1 (TTF1) is a immunohistochemical marker indicative of what primary?
Definition
thyroid and lung
Term
Chromogranin, synaptophysin, CD56 is a immunohistochemical marker indicative of what primary?
Definition
neuroendocrine tumors(e.g. SCLC)
Term
CDX-2 is a immunohistochemical marker indicative of what primary?
Definition
GI
Term
Calretinin is a immunohistochemical marker indicative of what primary?
Definition
mesothelioma
Term
Leukocyte common antigen is a immunohistochemical marker indicative of what primary?
Definition
lymphoma
Term
HMB-45, tyrosinase are immunohistochemical markers indicative of what primary?
Definition
Melanoma
Term
URO-III, thrombomodulin are immunohistochemical markers indicative of what primary?
Definition
bladder cancer
Term
WT-1 is a immunohistochemical marker indicative of what primary?
Definition
ovarian cancer
Term
CD 10 immunohistochemical marker indicative of what primary?
Definition
RCC
Term
what is the median survival of patients with disseminated carcinoma of unknown primary?
Definition
6-10 months
Term
which 6 malignancies are associated with hypercalcemia d/t secretion of PTHrP?
Definition
SCC (head and neck, skin, lung), breast, GU, GI
Term
which malignancy causes hypercalcemia d/t 1,25 dihydroxyvitamin D secretion?
Definition
lymphoma
Term
which 2 malignancies cause hypercalcemia d/t PTH secretion
Definition
lung, ovary
Term
which 2 malignancies cause hypercalcemia d/t PGE2 secretion?
Definition
renal, lung
Term
which 5 malignancies cause SIADH by secreting vasopressin?
Definition
Lung (squamous, small cell), GI, GU, ovary
Term
which 7 malignancies cause Cushing's syndrome by secretion of ACTH?
Definition
Lung (small cell, bronchial carcinoid, adenocarcinoma, squamous), thymus, pancreatic islet, medullary thyroid carcinoma
Term
which 4 malignancies cause cushing's syndrome by CRH secretion?
Definition
Pancreatic islet, carcinoid, lung, prostate
Term
which 6 malignancies cause hypoglycemia by secretion of insulin growth factor 2 (IGF2)?
Definition
sarcomas, adrenal, hepatic, GI, kidney, prostate
Term
which malignancy causes hypoglycemia by secretion of insulin?
Definition
small cell carcinoma of cervix
Term
which malignancies cause male feminization by bHCG secretion
Definition
hepatic, lung, pancreas, germ cell tumors
Term
which 4 malignancies cause diarrhea by secretion of calcitonine?
Definition
Lung, colon, breast, medullary thyroid carcinoma
Term
which 3 malignancies cause diarrhea by secretion of VIP?
Definition
Pancreas, pheochromocytoma, esophagus
Term
which malignancies cause oncogenic ostemalacia by secretion of FGF23?
Definition
Hemangiopericytomas, osteoblastomas, fibromas, sarcomas, giant cell tumors, prostate, lung
Term
which 2 malignancies cause acromegaly by secretion of GHRH?
Definition
pancreas, carcinoid
Term
which 2 malignancies cause acromegaly by secretion of GH?
Definition
pancreas, lung
Term
which 3 malignancies cause hyperthyroidism by secretion of TSH?
Definition
Hydatidiform mole, embryonal tumors, struma ovarii
Term
which 4 malignancies cause hypertension by secretion of renin?
Definition
kidney, lung, pancreas, ovary
Term
when should dialysis be used in the Tx of humeral hypercalcemia of malignancy?
Definition
when bisphosphonates and saline have too slow an onset
Term
what is a distinct Tx for humeral hypercalcemia of malignancy in the case of MM, lymphoma or leukemia?
Definition
glucocorticoids
Term
what is the treatment of malignancy associated SIADH in the patient with severe hyponatremia Na<115?
Definition
hypertonic saline 3% + fusid
Term
3 treatment options for malignancy associated SIADH?
Definition
1. water restriction
2. Demeclocycline
3. Conivaptan - V2 antagonist
Term
what is the MC malignancy to cause ectopic ACTH secretion?
Definition
SCLC
Term
8 symptoms that are distinct to cushing syndrome cause by ectopic malignant secretion?
Definition
fluid retention, hypertension, hypokalemia, metabolic alkalosis, poor wound healing, glucose intolerance, steroid psychosis, opportunistic infections
Term
which malignancy causing ectopic ACTH secretion is distinct in the fact that it may experience negative feedback by dexamethasone or metyrapone?
Definition
carcinoid
Term
which opportunistic infections attach the patient with ectopic ACTH secretion related malignancy?
Definition
mycoses, PCP
Term
what is the Tx of malignancy associated ACTH secretion?
Definition
1. metyrapone, ketoconazole
2. if resection of primary not possible, sometimes adrenalectomy is favorable
Term
what is a characteristic of a tumor that secretes IGF2 and causes hypoglycemia?
Definition
usually over 10 cm
Term
when does hypoglycemia occur in the IGF2 secreting tumor?
Definition
during fasting (night time)
Term
what are the signs of IGF2 induced hypoglycemia?
Definition
low serum glucose and insulin
IGF2 need not be elevated in serum
IGF2 mRNA elevated
Term
what is the Tx of IGF2 induced hypoglycemia?
Definition
treatment of underlying malignancy
iv glucose during sleep
glucagon and corticosteroids
Term
what are 3 malignancies that cause erythrocytosis by secretion of EPO?
Definition
Renal cancers
Hepatocarcinoma
Cerebellar hemangioblastomas
Term
5 malignancies that cause granulocytosis by secretion of G-CSF, GM-CSF or il6?
Definition
Lung cancer, Gastrointestinal cancer, Ovarian cancer, Genitourinary cancer, Hodgkin's disease
Term
5 malignancies that cause thrombocytosis by secretion of il6?
Definition
Lung cancer, Gastrointestinal cancer,
Breast cancer, Ovarian cancer, Lymphoma
Term
3 malignancies which cause eosinophilia by secretion of il5?
Definition
Lymphoma Leukemia Lung cancer
Term
7 malignancies which cause thrombophlebitis d/t to an unknown agent?
Definition
Lung cancer Pancreatic cancer Gastrointestinal cancer Breast cancer
Genitourinary cancer
Ovarian cancer Prostate cancer Lymphoma
Term
what is the Tx of malignancy associated erythrocytosis?
Definition
resection of tumor. if tumor not resectable, phlebotomy is indicated
Term
what % of patients with a solid tumor have granulocytosis?
Definition
30%
Term
what are the symptoms and Tx of malignancy associated granulocytosis?
Definition
asymptomatic, no need for Tx
Term
what are the symptoms of malignancy associated thrombocytosis? what is the Tx?
Definition
it is usually asymptomatic, not correlated to thrombosis of malignancy, hence no Tx needed. it is associated, however, with poorer prognosis
Term
what is a possible symptom of malignancy related eosinophilia? what is the Tx?
Definition
eosinophilia may lead to shortness of breath by infiltrating the lungs. this may be seen on CXR. palliative Tx: glucocorticoids
Term
what % of patients who develop DVT end up having cancer?
Definition
15%
Term
what is the risk for DVT in a patient with cancer undergoing surgery requiring general anesthesia?
Definition
30%
Term
what is the Tx for patients with cancer and proximal DVT and a relative contraindication to heparin anticoagulation (hemorrhagic brain metastases or pericardial effusion)?
Definition
placement of a filter in the inferior vena cava (Greenfield filter)
Term
what is an indication for placement of a filter in the inferior vena cava (Greenfield filter) in cancer patients?
Definition
proximal DVT and CIs to heparin anticoagulation (hemorrhagic brain metastasis or pericardial effusion)
Term
what are the 2 indications for anticoagulant prophylaxis in patients with cancer?
Definition
breast cancer undergoing chemotherapy
indwelling catheters
Term
what are 2 neurologic paraneoplastic syndromes caused by the oncoprotein anti-Hu? and in what malignancy?
Definition
Encephalomyelitis, subacute sensory neuronopathy - SCLC
Term
what paraneoplastic neurologic disease is caused by the oncoprotein anti-Yo? in which 2 malignancies?
Definition
Cerebellar degeneration - Ovary, Breast
Term
what 2 paraneoplastic neurologic diseases are caused by the oncoprotein anti-Ri, in what 3 malignancies?
Definition
Cerebellar degeneration, opsoclonus - breast, gynecologic, SCLC
Term
what paraneoplastic neurologic disease is caused by the oncoprotein anti-Tr? in which malignancy?
Definition
Cerebellar degeneration - hodgkin's
Term
what 5 paraneoplastic neurologic diseases are caused by the oncoprotein Anti-CV2/CRMP5? in what 2 malignancies?
Definition
Encephalomyelitis, chorea, optic neuritis, uveitis, peripheral neuropathy
SCLC, thymoma
Term
what 3 paraneoplastic neurologic diseases are caused by the oncoprotein Anti-Ma proteins? in what malignancy?
Definition
Limbic, hypothalamic, brainstem encephalitis
testicular cancer
Term
what 2 paraneoplastic neurologic diseases are caused by the oncoprotein Anti-amphiphysin? in what 2 malignancies?
Definition
Stiff-person syndrome, encephalomyelitis
Breast, SCLC
Term
what paraneoplastic neurologic disease is caused by the oncoproteins Recoverin and bipolar cell antibodies? in what 2 malignancies?
Definition
Cancer-associated retinopathy
Term
what 2 paraneoplastic neurologic diseases are caused by the oncoprotein Anti-GAD? in what malignancy?
Definition
Stiff-person, cerebellar syndromes
thymoma
Term
what 11 neurologic paraneoplastic diseases are associated with SCLC?
Definition
Encephalomyelitis, subacute sensory neuronopathy, Cerebellar degeneration, opsoclonus, Encephalomyelitis, chorea, optic neuritis, uveitis, peripheral neuropathy, Cancer-associated retinopathy, Stiff-person syndrome
Term
what 4 neurologic paraneoplastic diseases are associated with breast cancer?
Definition
Stiff-person syndrome, encephalomyelitis, Cerebellar degeneration, opsoclonus
Term
what % of patients with paraneoplatic neuronal disease of the CNS and PNS are found to have antibodies against neuronal or neuromascular proteins that may be diagnostic of the paraneoplastic phenomenom?
Definition
CNS - 60%
PNS - 20%
Term
what is the role of MRI and CSF studies in the diagnosis of paraneoplastic neuronal disease?
Definition
MRI and CSF are none specific except in the case of limbic encephalitis where you might see abnormalities in the mesial temporal lobes seen also only in non paraneoplastic limbic encephalitis and HHV6 infection. MRI and CSF are used to R/O spread of the cancer to the brain (metastasis)
Term
what is the general unproven treatment for most neurologic paraneoplastic diseases?
Definition
IVIG, corticosteroids, plasmapheresis
Term
Paraneoplastic Opsoclonus-Myoclonus Syndrome is usually associated with what 3 malignancies?
Definition
breast, lung and neuroblastoma in children
Term
what triggers the paraneoplastic stiff-person's syndrome? in what conditions do the symptoms disappear?
Definition
sensory or emotional trigger
symptoms disappear during sleep or general anesthesia
Term
what is the Tx of stiff person's syndrome?
Definition
1. Tx of the underlying tumor
2. glucocorticoids
3. gaba'ergic agents: diazepam, baclofen, sodium valproate, tiagabine, vigabatrin
Term
what is the treatment of Waldenström's Macroglobulinemia associated paraneoplastic peripheral neuropathies?
Definition
plasma exchange, IVIg, chlorambucil, cyclophosphamide, fludarabine, or rituximab
Term
which disease may involve the thymus in sjogren's syndrome?
Definition
MALT lymphoma
Term
which 5 diseases may involve the thymus?
Definition
castleman's, germ cell tumors, carcinoid, MALT lymphoma, thymoma
Term
what is the MCC of anterior mediastinal mass in adults?
Definition
thymoma
Term
what autoimmune systemic diseases are associated with thymoma?
Definition
myasthenia gravis, pure red cell aplasia, hypogammaglobulinemia (Good's syndrome), and less so UC, SLE, sjogrens, polymyositis etc.
Term
what % of thymoma patients suffer a concurrent systemic disease?
Definition
70%
Term
MRI has a defined role in the staging of posterior mediastinal tumors, what about anterior mediastinum?
Definition
nope
Term
what are the means of attaining staging in thymoma?
Definition
surgical mostly with the help of CT and somatostatin labeled imaging for local invasion
Term
what is the main principle in the staging of thymoma?
Definition
invasiveness
Term
what % of thymomas are noninvasive?
Definition
65%
Term
how can prognosis, which is measured by the degree of invasiveness of the thymic tumor, be anticipated?
Definition
it correlates with the histology type: thymomas are good, thymic carcinoma are bad
Term
what is the unique Tx step for stage 3 and 4 - invasion of neighboring organs, dissemination to pleura/pericard?
Definition
adjuvant chemotherapy
Term
which thymoma associated autoimmune systemic disease doesn't benefit from thymectomy at all?
Definition
hypogammaglobulinemia
Term
what is a long term complication on the eyes associated with the use of Radiation?
Definition
cataract
Term
3 long term complications of the heart associated with the use of Radiation?
Definition
Pericarditis, myocarditis, coronary artery disease
Term
4 long term complications of the heart associated with the use of chemotherapy?
Definition
dysrhythmias, cardiomyopathic congestive heart failure (CHF), pericardial disease, and peripheral vascular disease
Term
what are 5 cofactors in promoting anthracycline-associated CHF
Definition
Advanced age, other concomitant cardiac disease, hypertension, diabetes, and thoracic radiation therapy
Term
what is the 2nd most cardiotoxic chemotherapeutic agent after anthracyclines?
Definition
Trastuzumab (Herceptin)
Term
how is cardiotoxicity d/t chemotherapy monitored?
Definition
1. MUGA - multigated acquisition scan (checks ejection fraction via nuclear-bound RBCs)
2. US
Term
how does cardiotoxicity caused by Trastuzumab differ from that by anthracyclines?
Definition
reversible, not dose-dependent, no permanent effect on myofibrils
Term
which test monitors for Bleomycin pulmonary toxicity and recovery from toxicity?
Definition
Diffusion capacity of the lungs for carbon dioxide (DLCO)
Term
what are 5 additive risk factors to bleomycin induced pulmonary pneumonitis?
Definition
age, prior lung disease, concomitant use of other chemotherapy, lung irradiation and high FiO2
Term
what are risk factors for radiation pneumonitis?
Definition
advanced age, poor performance status, preexisting compromised pulmonary function, radiation volume, and dose
Term
what is The dose "threshold" for radiation pneumonitis?
Definition
range of 5-20 Gy
Term
what is the Tx of radiation/chemotherapy induced pneumonitis?
Definition
steroids
Term
which is the only chemotherapeutic agent causing pneumonitis that does not respond to steroid Tx?
Definition
nitrosoureas
Term
Neurocognitive dysfunction, "chemo brain", in children with ALL is d/t which chemotherapeutic agents
Definition
intrathecal MTX + Cytarabine and radiation therapy to the cranium
Term
what is the pathophysiology of early onset brain radiation toxicity vs. late onset?
Definition
reversible demyelination
focal necrosis
Term
what is a symptom of spinal cord radiation toxicity?
Definition
Lhermitte's sign - electrical shocks up the spine
Term
what is a Tx to prevent cholestasis in the setting of high-dose chemotherapy before bone marrow transplant?
Definition
Ursodiol - reduces cholesterol absorption and is used to dissolve (cholesterol) gallstones
only FDA approved Tx for primary billiary cirrhosis
Term
which 4 organs are at the highest risk to develop secondary cancer d/t radiation?
Definition
thyroid, breast, sarcomas, CNS cancers
Term
what 2 malignancies are mainly associated with chemotherapeutic treatment?
Definition
acute leukemia and MDS
Term
what are the chromosomal characteristics of acute leukemia secondary to alkylating agents?
Definition
deletions in chromosomes 5 or 7
Term
what are the chromosomal characteristics of acute leukemia secondary to antitopoisomerase agents?
Definition
t(10:23)
Term
which malignancy is associated with treatment causing secondary bladder cancer?
Definition
non-hodgkin's lymphoma
Term
how do you distinguish iron deficiency anemia from the thallasemias
Definition
thallasemias: normal or increased serum iron and transferin saturation, low RDW
Term
how do you distinguish myelodysplastic syndromes from iron deficiency anemia - both cause microcytic hypochromic anemia
Definition
myelodysplastic syndromes - high serum iron
Term
what is the association btwn endocrine function and anemia
Definition
thyroid, cortisol, testosterone deficiency can cause anemia

it is important to remember when correcting cortisol levels d/t addison's disease to also make sure thyroid function is normal otherwise anemia may drastically worsen
Term
2 indications for iron transfusions
Definition
serious underlying cardiovascular or pulmonary disease - 11 g/dl

otherwise - 8 g/dl
Term
3 general elements in The management of acute painful sickle cell crisis
Definition
1. vigorous hydration,
2. thorough evaluation for underlying causes (such as infection), and
3. aggressive analgesia
Term
2 indications for treatment with hydroxyurea in sickle cell anemia
Definition
in patients experiencing repeated episodes of acute chest syndrome or with more than three crises per year requiring hospitalization
Term
indications for BMT in children with sickle cell anemia
Definition
presence of repeated crises early in life, a high neutrophil count, or the development of hand-foot syndrome
Term
prophylactic treatment of stroke in children with sickle cell anemia
Definition
exchange transfusion
Term
skeletal abnormalities seen in the thallasemias
Definition
"chipmunk" facies due to maxillary marrow hyperplasia and frontal bossing. Thinning and pathologic fracture of long bones and vertebrae
Term
when is splenectomy required in thallassemia
Definition
if the annual transfusion requirement (volume of RBCs per kilogram of body weight per year) increases by >50%
Term
how is the diagnosis of thallasemia made
Definition
severe anemia accompanied by the characteristic signs of massive ineffective erythropoiesis: hepatosplenomegaly, profound microcytosis, a characteristic blood smear (tear drop cells), and elevated levels of HbF, HbA2
Term
Children with severe homocystinuria (blood levels 100 mol/L) due to deficiency of one of three enzymes, methionine synthase, MTHFR, or cystathionine synthase (Fig. 105-1), have vascular disease, e.g., ischemic heart disease, cerebrovascular disease, or pulmonary embolus as teenagers or in young adulthood
Definition
Term
prophylactic folic acid in pregnancy lowers the risk of what malignancy in childhood
Definition
ALL
Term
5 GI conditions associated with decreased absorptions of B12
Definition
Simple atrophic gastritis (food cobalamin malabsorption)

Zollinger–Ellison syndrome

Gastric bypass surgery

Use of proton pump inhibitors

Celiac disease
Term
7 substances that are associated with reduced absorption of Vit B12
Definition
colchicine,

neomycin, (aminoglycoside)

slow-release potassium chloride,

anticonvulsant drugs,

metformin,

cytotoxic drugs

Alcohol
Term
first line therapy of AIHA? second line? 3rd?
Definition
1st: GCS
2nd: Rituximab or splenectomy
3rd: Azathioprine, cyclophosphamide, cyclosporine, and IVIG
Term
Paroxysmal Cold Hemoglobinuria (PCH)?
Definition
rare form of AIHA; children;viral infection;Donath-Landsteiner antibody;binds at low temps and lysis RBCs back at body temp
Term
what is the mechanism of hemolytic anemia caused by septicemia
Definition
Exotoxins produced by Clostridium perfringens
Term
7 conditions causing intravascular hemolytic anemia
Definition
Mismatched blood transfusion
Paroxysmal nocturnal hemoglobinuria (PNH)
Paroxysmal cold hemoglobinuria (PCH)
Septicemia
Microangiopathic
March hemoglobinuria
Favism
Term
what is the clinical triad of PNH (not always present)
Definition
hemolysis, pancytopenia and venous thrombosis
Term
PNH is associated with what other hematologic condition
Definition
aplastic anemia
Term
what is the gold standard for the Dx of PNH
Definition
flow cytometry showing a population of RBCs or granulocytes that are CD59-, CD55-
Term
2 main treatments for PNH
Definition
recurrent transfustions

eculizumab, antibody directed against the complement component C5
Term
2 synthetic oxygen carriers which are studied to be used as universal replacements to all blood supplements
Definition
hemoglobin-based oxygen carriers

fluorocarbon synthetic chemicals
Term
5 infections known to cause pancytopenia
Definition
Brucellosis

Tuberculosis

Leishmaniasis

Q fever

Legionnaires' disease
Term
4 viruses that can cause aplastic anemia
Definition
Epstein-Barr virus (infectious mononucleosis)
Hepatitis (non-A, non-B, non-C hepatitis)
Parvovirus B19 (transient aplastic crisis, PRCA)
HIV-1 (AIDS)
Term
what is the most common early symptom of aplastic anemia
Definition
bleeding
Term
what does the biopsy in aplastic anemia show
Definition
fat
Term
when is isolated thrombocytopenia an indication for bone marrow biopsy
Definition
patients older than 60 b/c of the risk for myelodysplasia
Term
what is the initial step in the workup of thrombocytopenia?
Definition
check for abnormalities of RBCs and WBC on CBC
Term
what is the next step in the workup of thrombocytopenia, after it has been established that CBC is otherwise normal?
Definition
blood smear
Term
what does fragmented RBCs on blood smear in a patient with isolated thrombocytopenia mean?
Definition
microangiopathic hemolytic anemias (TTP, DIC)
Term
what is the next step in the workup of thrombocytopenia in a patient with other abnormalities on CBC
Definition
bone marrow examination
Term
9 drugs that cause isolated thrombocytopenia most frequently
Definition
heparin
Furosemid
Gold, used to treat arthritis
Nonsteroidal anti-inflammatory drugs (NSAIDs)
Penicillin
Quinidine
Quinine
Ranitidine
Sulfonamides
Term
heparin induced thrombocytopenia is a thrombogenic state!
Definition
Term
which is more prevalent in HIT, Clexane or UFH
Definition
UFH
Term
what is the average exposure to heparin in HIT
Definition
5-14 days
Term
what is an important step in the management of HIT
Definition
preform lower extremity duplex Dopplers for DVT
Term
how is the diagnosis of ITP made?
Definition
exclusion of other causes of isolated thrombocytopenia: HIV infection and hepatitis C (and other infections if indicated); serologic testing for SLE, serum protein electrophoresis, and immunoglobulin levels to potentially detect hypogammaglobulinemia; selective testing for IgA deficiency or monoclonal gammopathies and, if anemia is present, direct antiglobulin testing (Coombs test) to rule out combined autoimmune hemolytic anemia with ITP (Evans syndrome)
Term
when is bone marrow examination indicated in the diagnosis workup of ITP?
Definition
patients older than 60
Term
which patients with ITP are not indicated for treatment
Definition
asymptomatic with platelets > 30,000
Term
what is the treatment of ITP without significant bleeding symptoms, severe thrombocytopenia (<5000/L), or signs of impending bleeding (such as retinal hemorrhage or large oral mucosal hemorrhages)? what is the treatment if those exist?
Definition
outpatient, monotherapy with: GCS, IVIG, anti-Rh(D) in Rh-positive patients only b/c you want to create hemolysis

inpatient: GCS + one of the other options
Term
what is the role of splenectomy in the treatment of patients with ITP
Definition
relapse after glucocorticoids are tapered
Term
2 novel treatments of ITP
Definition
romiplostim and eltrombopag
Term
what is the mainstay of treatment of TTP
Definition
Plasma exchange
Term
what is the treatment of von Willebrand Disease?
Definition
DDAVP (vasopressin) and e-aminocaproic acid or tranexamic acid
Term
what is an alternative treatment to factor replenishment in Hemophilia A
Definition
vasopressin DDAVP
Term
what is the test to check for factor inhibitors (antibodies) in hemophilia
Definition
bethesda - add plasma to patients serum and see if coagulation occurs or not
Term
what is the indication for treatment in FXI def.
Definition
patients with severe disease required to undergo a surgical procedure
Term
which substances are known to cause DIC
Definition
Fibrinolytic agents
Aprotinin
Warfarin (especially in neonates with protein C deficiency)
Prothrombin complex concentrates
Recreational drugs (amphetamines)
Term
what is the treatment of DIC
Definition
FFP - 1 unit of FFP increases most coagulation factors by 3% in an adult without DIC
Term
what is the typical presentation of autoimmune hemolytic anemia
Definition
The onset of AIHA is very often abrupt and can be dramatic. The hemoglobin level can drop, within days, to as low as 4 g/dL; the massive red cell removal will produce jaundice; and sometimes the spleen is enlarged
Term
what is the presentation of CML?
Definition
leukocytosis, <5% blasts in peripheral blood, elevated platelets count
Term
what is the medical treatment of aplastic anemia in case HSCT is not an option
Definition
antithymocyte Ab + cyclosporine
Term
what is the treatment of coumadin overdose?
Definition
asymptomatic INR 3.5-4.5: stop coumadin
asymptomatic INR >4.5 : stop coumadin + give vitamin K
bleeding - FFP
Term
2 malignancies associated with PTHrP
Definition
RCC and SCC of lung
Term
cyclophosphamide - side effects
Definition
cystitis + pulmonary fibrosis
Term
MC malignancy post renal transplantation
Definition
NHL
Term
what helps absorb iron
Definition
vit C
Term
what type of kidney injury does cisplatin cause
Definition
ATN
Term
what type of kidney injury does cisplatin cause
Definition
ATN
Term
what type of kidney injury does cisplatin cause
Definition
ATN
Term
what type of kidney injury does cisplatin cause
Definition
ATN
Term
טיפול בברזל מיועד ל...
Definition
חולים שאינם מסוגלים לסבול ברזל אוראלית, יש להם חסר חמור, או שהם נדרשים למתן ברזל על בסיס מתמשך
Term
in the physical examination of MM, what distinguishes it from the rest of the plasma cell disorders?
Definition
no hepatosplenomegaly nor lymphadenopathy
Term
what is the MCC of renal failure in MM
Definition
hypercalcemia
Term
MM can have IgM
Definition
Term
MGUS occurs in 10% of population over 75
Definition
Term
causes of cold agglutination hemolytic anemia
Definition
lymphoma, CLL, mycoplasma, HIV, mononucleosis
Term
causes of warm agglutination hemolytic anemia
Definition
CLL, ovarian teratoma, connective tissue disease–eg, SLE, progressive systemic sclerosis, rheumatoid arthritis, ulcerative colitis
Term
hairy cell leukemia
Definition
oval nuclei, abundant cytoplasm, distinctive membrane projections, pancytopenia, splenomegaly, tartrate-resistant acid phosphatase, dry tap, monocytopenia - TB, splenectomy, CDA treatment
Term
pancytopenia 6 years after breast cancer
Definition
MDS
Term
how to distinguish PV from Relative erythrocytosis
Definition
RBC mass
Term
rbc count - Iron def vs. thallasemia
Definition
low vs high (5)
Term
what is the most common transfusion reaction
Definition
febrile non hemolytic transfusion reaction
Term
what is the mechanism of febrile non hemolytic transfusion
Definition
Antibodies directed against donor leukocyte and HLA antigens
Term
fever + skin lesions resembling erythema multiforme in neutropenic cancer patients = sweet's syndrome. treatment?
Definition
GCS
Term
necrotic skin lesions in immunosuppressed cancer patients may be caused by unusual pathogens such as E.Coli, pseudomonas or candida
Definition
Term
what is the role of smoking and alcohol in the risk of pancreatic cancer
Definition
smoking is a risk factor, alcohol isn't
Term
MDS prognosis
Definition
depends on % of blasts
Term
what can be seen on blood smear of MDS
Definition
few if any blasts
macrocytosis, no granules in platelets and neutrophils
Term
what can be seen in the BM of MDS
Definition
normal or hypercellular (20% - hypocellular), little megakaryocytes
Term
PNH triad
Definition
intravascular hemolysis, pancytopenia, venous thrombosis
Term
treatment of factor 11 def.
how does it manifest itself?>
Definition
only before surgery
menorrhea, epistaxis
Term
sideroblastic anemia
Definition
excess iron - HSM, liver and kidney damage
Term
what is the treatment of sideroblastic anemia
Definition
Vit B6, blood transfusion, BM transplant. do not give EPO
Term
abnormalities that cause venous thromboembolism (vs. arterial)
Definition
protein C,S def., factor V leiden, hyperprothrombin, anti-thrombin 3 def.
Term
abnormality that causes arterial thromboembolism
Definition
hyperhomocysteinemia
Term
anti thrombin 3 def.
Definition
venous, high dose heparin, lifetime anti-coag after 1 event of PE/DVT, OCP CIed, prophylactic anticoagulation in pregnancy and before surgery
Term
dry tap
Definition
CML, myelofibrosis, hairy cell leukemia, acute leukemia, hodgkin's lymphoma, iron def/pernicious/hemosiderin anemia, metastasis to BM
Term
thrombocytopenia in alcoholics
Definition
injury to BM + folate def
Term
viral thrombocytopenia
Definition
HBV, HIV, HCV, CMV
Term
drugs that cause thrombocytopenia
Definition
thiazide, phenytoin, chemo, vancomycin, sulfa, linezolid, ACEi
Term
CML doesn't cause hemolytic anemia
Definition
Term
anti thrombin 3 def is a life-long indication for anticoagulation
Definition
Term
when is MM associated with increased ALP
Definition
only in bone fractures (not just lytic bone lesions)
Term
anti thrombin 3 def
Definition
lifelong coumadin, clexane during whole pregnancy, no need for aspirin, test family members, avoid oral contraceptives
Term
O2-Hb dissociation curve
Definition
right shift - O2 delivered more deliberately to tissues: acidosis and temperature elevation
Term
Vit B12 elevated in CML
Definition
Term
3 indications to treat in CLL (Fludarabine)
Definition
1. Hb<10
2. PLT<100,000
3. >3 areas + [symptoms or lymphadenopathy]
Term
indications for surgery in stage 3A NSCLC
Definition
מעורבות בלוטות לימפה איפסילטרליות פריברונכיאליות ו/או הילריות נודולות בתוך הריאות שהגיעות בהתפשטות ישירה מהגידול הראשוני
Term
duration of anticoagulation in DVT
Definition
unprovoked - lifelong
PE, post-surgical, proximal leg - 6 months
distal leg - 3 months
Term
דיספיברינוגנמיה
Definition
• מחלות של הפרעות בפיברינוגן, בעיקר AD שמתאפיינות בדימום ולעיתים טרומבוזיס.
• מתגלה לפעמים עקב הפלות חוזרות, בעיות בריפוי פצע, דימום, טרומבוזיס.
• לרוב אסימפטומטי
• במעבדה עליה בPT , עליה TT ופיברינוגן נורמלי.
• יכול להופיע גם במחלות כבד עקב הפרעה בפולמריזציה של פיברין
Term
הטיפול בחוסר ויטמין B12
Definition
• B12 לשריר כל 3-7 שבועות במשך 6 פעמים למילוי מאגרים
• לאחר מכן כל 3 חודשים, למרות שנהוג כל חודש
Term
פוליציטמיה ורה - היפראוריצמיה וביופסיית מח עצם
Definition
• היפראוריצמיה , גאוט ואבני חומצה אורית
• מח עצם – לא מספק מידע אבחנתי כי יכול להיות נורמלי או כזה שלא ניתן להבדיל מIMF או ET. לכן, אלא אם כן יש לבסס אבחנה של מילופיברוזיס או לשלול מחלות אחרות, אין צורך לבצע.
Term
הסיכון לממאירות שנייה לאחר טיפול בהודגקין
Definition
o ב10 שנים ראשונות יש סיכוי מוגבר ללוקמיות חריפות- עקב כימותרפיה , יותר בMOPP והקרינה
o לאחר 10 שנים סיכוי מוגבר לקרצינומות קשור יותר לחשיפה לקרינה.כולל סרטן שד וריאה בשדה קרינה ולכן יש צורך בסקירה והפסקת עישון
Term
דימומים ב-AML
Definition
o דימומים נפוצים יותר ב APL או ב Monocytic AML אלו גם נוטים לפלוש יותר לרקמות כמו חניכיים, עור וכד' (איפה שמונוציטים אוהבים ללכת)
Term
שינויים ציטוגנטיים טובים ב-AML
Definition
t15:17(APL), t8:21, inv16
Term
שינויים ציטוגנטיים גרועים ב- AML
Definition
t6:9, inv3/7
Term
ת"ל של Cytarabine – פגיעה צרבלרית
Definition
Term
CLL במי מטפלים
Definition
o חולה B-cell CLL עם מעורבות של מח העצם ולימפוציטוזיס בלבד ללא הפרעות נוספות- מעקב בלבד. השרדות- 10 שנים.
o ב-intermediate stage (לימפאדנופתיה ו/או הפטופלנומגליה) עם ספירה תקינה ואסימפטומטי- בתחילה מעקב וכעבור מספר שנים- טיפול. השרדות- 7 שנים.
o כשל מח עצם- טיפול! השרדות ללא טיפול- 1.5 שנים.
Term
השלב המואץ של CML מוגדר על ידי....
Definition
אנמיה שלא ניתן להסביר על ידי טיפול או דימום, ציטוגנטיקה קלונאלית ו10-20 אחוז בלסטים בדם או במח עצם או 20 אחוז בזופילים בדם או מח עצם או טסיות מתחת ל100000
Term
הטיפול בסימפטומים ההמורגיים של DIC
Definition
FFP אם PT מעל 1.5, רמות נמוכות של פיברינוגן או היפרפיברונלוזיה מהירה יכול לדרוש קריו (עשיר בפקטור 8, פיבירונוגן VWF), טסיות
Term
טיפול בסימפטומים של קרישתיות יתר ב-DIC
Definition
הפרין יכול להנתן במינון נמוך בחולים עם מחלה קלה על רקע APL או גידול סולידי או טרומבוזיס ברור. כמו כן בפורפורה פולמינס, כריתת המנגיומה והוצאת עובר מת. אך במחלה אקוטית הפרין יחמיר דימום. חסר בפרוטין C –ניתן להשלים ולתת אותו מבחוץ אם זו הסיבה לפרופורה פולמיננס.
Term
הסימפטום הכי נפוץ באנמיה אפלסטית
Definition
דימום - מח ועין
Term
אנמיה אפלסטית קשה נקבעת ע"י שניים מהבאים:
Definition
נויטרופניה של 500 ומטה, טסיות מתחת ל20000, רטיקולוציטים מתחת לאחוז
Term
איך לטפל בחולה שמקבל קומדין וצריך לעבור בדיקה פולשנית
Definition
• יש להפסיק את הטיפול בקומדין 5 ימים טרם הטיפול החודרני כדי שINR יחזור לנורמלי
• למי שבסיכון גבוה לטרומבוזיס ניתן לקבל קלקסן, כאשר הINR נופל מתחת ל2
• המינון האחרון של קלקסן ינתן 12-24 שעות טרם הפעולה
• לאחר הפעולה ניתן לשוב לטפל בקומדין
Term
מה יהפוך בטא תלסמיה מיגור לקלה יותר קלינית
Definition
נשאות ל-a-Thalassemia במקביל- חומרת ביתא תלסמיה מייג'ור נובעת מעודף שרשראות אלפא. לכן נשאות לאלפא תלסמיה תקל על חומרת המחלה כי יש פחות חוסר איזון

-Globin gene chains can substitute for chains, generating more
hemoglobin and reducing the burden of -globin inclusions ייצור פרסיסטנטי של שרשאות גמא-גלובין.

מוטציה הגורמת לפגיעה קלה יותר בייצור שרשראות בתא-גלובין
Term
אנמיה המוליטית בשל ספרוציטוזיס
Definition
MCHC גבוה
Term
תאי דם אדומים חרמשיים במשטח וטחול מוגדל=אנמיה חרמשית + טלסמיה. אם הטחול אינו מוגדל=אנמיה חרמשית טהורה
Definition
Term
in which malignancies is Cetuximab used (EGFR antibody)?
Definition
CRC - advanced and mets
Head and neck - advanced and mets
NSCLC - advanced
Term
in which malignancies is erlotinib (Tarceva) used?
Definition
metastatic pancreas - with gemcitabine
HCC- stage 3 and 4 with avastin
NSCLC - 2nd line advanced disease, maintenance
Term
in which malignancies is etoposide used
Definition
nonseminomas stage 2, ewing sarcoma, SCLC - with platinum
Term
in which malignancies is paclitaxel used?
Definition
2nd line met breast cancer - monotherapy/avastin
TCC - metastatic with gemcitabine and cisplatin
NSCLC - advanced, with cituximab/avastin/cisplatin
head and neck - metastatic
Term
Nexavar (sorafenib)
Definition
RCC - metastatic 1st line
stage 3 and 4 HCC - Multiple unilobar tumors or tumor with vascular invasion, Extrahepatic HCC or elevated bilirubin
Term
avastin
Definition
metastatic breast cancer
HCC - extrahepatic disease, with erlotinib
NSCLC - advanced disease that is non-squamous with caboplatin and paclitaxel
relapse of ovarian cancer
Term
when to treat CLL
Definition
Weakness, night sweats, weight loss, painful lymphadenopathy, or fever

Symptomatic anemia and/or thrombocytopenia


Autoimmune hemolytic anemia and/or thrombocytopenia poorly responsive to corticosteroid therapy

Progressive disease, as demonstrated by increasing lymphocytosis with a lymphocyte doubling time less than six months, and/or rapidly enlarging lymph nodes, spleen, and liver. In contrast, transient localized lymphadenopathy, occurring in response to localized infections, is not necessarily an indication for treatment.

Repeated episodes of infection. Hypogammaglobulinemia without repeated episodes of infection is not a clear indication for therapy
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