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Pathology
Pathology III
227
Pathology
Professional
04/06/2011

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Term
von Willebrand Factor is released from ____________ and acts to.............
Definition
Damaged endothelial cells and binds to exposed basement membrane to provide an adherent surface for platelets.
Term
______ is a receptor on the platelet that binds vWF.
Definition
GP1b

Bernard Soulier Syndrome is a defect in this receptor, thus the patient has prolonged bleeding times despite the PT and PTT being normal.

Slows the platelet to allowance adherence to ECM.
Term
Primary (temporary) hemostatic plug
Definition
Platelets releasing ADP, serotonin, and thromboxane A2 causing more platelets to aggregate.

CANNOT occur in patients with thrombocytopenia.
Term
Secondary (permanent) hemostatic plug
Definition
Endothelial cells produce tissue factor; coagulation cascade induces thrombin formation which then cleaves fibrinogen.

CANNOT occur in patients with hemophilia.
Term
Some of the ways in which endothelial cells block platelet aggregation......
Definition
1) Prostacyclin (PGI2)
2) NO
3) ADPase
Term
Some of the ways in which endothelial cells block the coagulation cascade......
Definition
1) Thrombomodulin- converts thrombin to an anticoagulant
2) Heparin- cofactor for anti-thrombin III which inactivates IXa and Xa.
Term
Platelet Factor 3
Definition
Catalyzes the conversion of prothrombin into thrombin.
Term
The release of alpha (larger) and delta (ADP) granules from platelets in controlled by.....
Definition
CAMP levels.

A rise in CAMP inhibits release while a fall in CAMP triggers release.
Term
Role of calcium in hemostasis
Definition
Activates phospholipases to generate prostaglandins; also augments the coagulation cascade.
Term
Platelet Factor 4
Definition
Neutralizes heparin
Term
Both the intrinsic and extrinsic coagulation cascade converge at......
Definition
Activating IX --> IXa which then acts with VIIIa (with Ca present) to activate X --> Xa.

People with hemophilia have a mutation in VIII or IX.
Term
The conversion of prothrombin to thrombin is mediated by......
Definition
Xa, V, and platelet factor 3.

The clot is stabilized by XIII.
Term
Role of vitamin K in hemostasis
Definition
Required by liver microsomal carboxylase that modifies VII, IX, X, and prothrombin.

Needed by protein C (activated by thrombin in the presence of thrombomodulin); Proteins C and S degrade Va and VIIIa which help out thrombomodulin.
Term
Prothrombin time measures the ________ pathway.
Definition
Extrinsic pathway.....normal time is 12 seconds.
Term
Most common cause of thrombocytopenia.
Definition
Platelets become aggregated by a virus or bacteria and get trapped in the spleen.
Term
_____ is a receptor on the platelet that promotes aggregation.
Definition
GPIIb-IIIa

Thrombasthenia is a defect in this receptor leading to prolonged bleeding time despite the PT and PTT being normal.
Term
Hemophilia A versus Hemophilia B
Definition
Defect in VIII versus IX; strangely, only PTT is affected.

Hemophilia is an X-linked recessive disorder.
Term
______________ is the most common bleeding disorder.
Definition
Von Willebrand Disease; affects both platelet plug formation AND coagulation (due to Factor VIII dependency); increased bleeding time and PTT.

No bleeding into joints (hemarthrosis) as in hemophilia though.
Term
Virchow's Triad
Definition
Factors that promote thrombosis:

1) Endothelial injury
2) Abnormal blood flow
3) Hypercoagulability
Term
Trousseau's sign
Definition
Disseminated venous thrombosis in cancer patients; may be due to tumor releasing procoagulant factors.
Term
Propagated thrombosis
Definition
Thrombus is not mural and is almost being occluding; blood flow slows going around it; especially common in veins.
Term
Cor pulmonale
Definition
Greater than 50% of the pulmonary blood flow being blocked leading to acute right ventricular failure.

Slightly different than saddle embolus.
Term
The most common genetic cause of hypercoagulability is......
Definition
Factor V mutation causing it not to be inactivated by protein C.
Term
Transmural infarction
Definition
Myocardial necrosis that traverses the entire ventricular wall; unlike a subendocardial infarction.
Term
After an acute myocardial infarct, the affected area is yellow at Day __ and red at Day __.
Definition
3 (replacement of PMNs by macrophages).......10
Term
Which type of bacteria cause rheumatic fever?
Definition
Beta-hemolytic streptococci; causes body to attack cardiolipin.
Term
MacCallum plaque
Definition
Rheumatic endocarditis formed on the posterior wall of the left atrium.
Term
Mitral valve stenosis is marked by.....
Definition
Diastolic pressure higher in the left atrium than in the left ventricle; systolic output is affected.
Term
Acute endocarditis is caused by......
Definition
Staph aureus (50% of cases); Strep viridans causes subacute endocarditis.
Term
While tricuspid valve involvement is rare in rheumatic heart disease, is may be involved in _______________.
Definition
Endocarditis of the carcinoid syndrome.
Term
Pulmonary valve involvement is most often seen in ________________.
Definition
Congenital malformations (ex. tetralogy of Fallot); also may be involved in the carcinoid syndrome.
Term
Congenital rubella syndrome
Definition
Link between rubella in mother in FIRST TRIMESTER and cardiovascular defects, microcephaly, cataracts, and growth/mental retardation.
Term
Myocarditis
Definition
Most often presents as biventricular heart failure in young people with other heart problems; most often VIRAL.
Term
_____________ is the most frequently occuring cardiac tumor.
Definition
Myxoma of the left atrium.
Term
Right-sided CHF is most commonly caused by......
Definition
Left ventricular failure
Term
Tetralogy of Fallot
Definition
1) Pulmonary infundibular or stenodid
2) Ventricular septal defect
3) Overriding aorta
4) Right ventricular hypertrophy
Term
The outer half of the tunica media is oxygenated by the ___________.
Definition
Vaso vasora
Term
Fibrofatty plaques begin in the tunica __________ and contain _____________.
Definition
Intima.........necrotic debris.
Term
Less oxygenation in a necrotic core of an atheroma can create _____________.
Definition
Embolic showers
Term
Fusiform aneurysm
Definition
Cylindrical; due to atrophy of the media; common in abdominal aorta
Term
During atherogenesis, ___________ occurs first and then _____________ follows.
Definition
Monocyte adhesion and immigration into intima...........entry of smooth muscle cells.
Term
_________ marks the stage for fatty streaks in atherosclerosis.
Definition
Oxidized LDLs which are chemotactic for monocytes among other things; also cause endothelial cells to express adhesion receptors; also cause macrophages to express "scavenger receptors" which bind modified LDL (not downregulated like LDL receptors).
Term
____________ marks the stage for the fibrous plaque in atherosclerosis.
Definition
Smooth muscle cells secreting dermatan sulfate which preferentially binds extracellular LDL, thus contributing to its retention.
Term
Hyperlipidemia can increase the activity of ___________.
Definition
Platelets
Term
Homocysteine levels can be returned to normal in patients with enzymatic defects using _________.
Definition
Folic acid
Term
Response to Injury Hypothesis of Atherosclerosis
Definition
Chronic injury to the endothelium induces release of cytokines (ex. heat shock proteins) which then induce inflammation or recruit WBCs (autoimmunity).
Term
Normotension is defined as......
Definition
<140/<90
Term
Hypertension is defined as..........
Definition
>160/>95
Term
Autoregulation
Definition
The reflexive action of the vessel to narrow its lumen size to prevent hyperperfusion of sensitive tissues such as the CNS.
Term
Renal Rention Hypothesis of primary hypertension
Definition
Increase in Na retention in the kidney increases cardiac output which leads to autoregulation (bad).
Term
Vasoconstriction and Vascular Hypertrophy Hypothesis of primary hypertension
Definition
Increased peripheral resistance is the initiating event which eventually leads to smooth muscle cell proliferation in the vessels (mediated by Angiotensin II).
Term
Preeclampsia
Definition
Secondary hypertension during pregnancy; can be fatal, so ultimate treatment is to terminate the pregnancy.
Term
Hyaline arteriolosclerosis
Definition
Occurs with aging in normal individuals, but is far more prevalent in people with primary benign hypertension.

Hyperplastic ateriolosclerosis is associated with malignant hypertension; marked by reduplication of the basement membrane (onion skinning); can also cause necrotizing arteriolitis.
Term
Systemic hypertensive heart disease
Definition
Left-sided heart failure; marked by left ventricular/atrial hypertrophy with reduced tissue perfusion (especially to the kidneys); therefore, patient develops azotemia.
Term
Grading scale for retinopathy caused by hypertension.....
Definition
1) Generalized narrowing of arterioles
2) Grade I + focal arteriolar spasms
3) Grades I&II + flame-shaped hemorrhages and hard, waxy exudates
4) Grades I,II,&III plus optic disc edema (papilledema)
Term
Malignant nephrosclerosis
Definition
Kidney has petechial hemorrhages; arterioles progress to fibrinoid necrosis; kidney activates renin/angiotensin which further exacerbates the problem.

Hyperplastic arteriolosclerosis is a new finding...
Term
Malignant hypertension
Definition
>220/>140

Often a complication of BENIGN hypertension; death is due to uremia, stroke, or cardiac failure; we see medial thickening associated with infiltration of plasma components leading to fibrinoid necrosis; endothelial cells proliferate giving an "onion skin" appearance.

These patients will present with headache, nausea, vomiting, and scotomas (spots before the eyes) due to swelling in the brain and retina.
Term
Atrial natriuretic factor
Definition
Gets the kidneys to secrete Na thus lowering the blood volume.
Term
Liddle syndrome
Definition
Rare single-mutation genetic defect in sodium channel leading to Na retention.
Term
While the kidney is the major regulator of blood pressure, ___________ is the major responder.
Definition
Vessel wall
Term
The most common problem with hypertension is.........
Definition
Atherogenesis resulting in thrombosis!
Term
Which part of the vasculature carries out the main functions of autoregulation?
Definition
Arterioles
Term
Evidence of clonal origin in uterine tumors......
Definition
Uterine tumors express only one isozyme of G6PD unlike the mosaic in the normal uterus.
Term
All tumors have parenchyma and stroma (true/false).
Definition
True
Term
Benign tumors never express __________, _________, or ___________.
Definition
Dysplasia, anaplasia, or atypical mitosis.

May see some metaplasia though.
Term
Myoma
Definition
Benign smooth muscle tumor; rhabdomyoma is a benign skeletal muscle tumor.
Term
Sarcomas are of ___________ origin and include.....
Definition
Mesenchymal tissue........angiosarcoma and leiomyosarcoma.
Term
The "oma's" that are not benign.....
Definition
1) Melanoma
2) Hepatoma = hepatocellular carcinoma
3) Mesothelioma
4) Seminoma = malignant germ cell tumor
5) Teratoma: can be epithelial or mesenchymal; always malignant in the testes but can be benign in the ovaries.
Term
___________ derivatives are all epithelial in origin.
Definition
Foregut derivatives; includes liver, pancreas, gall bladder, stomach, diaphragm, cystic duct, and bile duct.

Are all carcinomas!
Term
___________ derivatives are of mesenchymal origin.
Definition
Neural crest; includes dorsal root ganglia, ANS, ganglia of cranial nerves V, VIII, IX, & X, Schwann cells, meninges of brain, melanocytes, adrenal medulla, head tissue, and tooth structures EXCEPT for enamel.
Term
Hamartoma vs. Choristoma
Definition
Hamartoma is the development of deformed blood vessels or other tissue that belongs in the area; a choristoma is the development of tissue that does not belong in the area.
Term
Mixed tumors are derived from.......
Definition
One germ layer but give rise to different phenotypes due to divergent differentiation of the neoplastic stem cell; examples include fibromyxoid stroma and mixed tumors of salivary glands.
Term
Teratomas are derived from......
Definition
More than one germ layer; rests of these cells (choristomas) are occasionally found in the midline giving rise to midline teratomas.
Term
Differentiation of a neoplasm refers to its (parenchyma/mesenchyma).
Definition
Parenchyma; determines the extent to which neoplastic cells resemble their forbears.
Term
Desmoplasia
Definition
The formation and proliferation of connective tissue in response to neoplastic growth; typical in breast and salivary tumors.
Term
___________ is a hallmark of malignancy in a tumor.
Definition
Anaplasia; represents primitive, more aggressive cells; marked by pleomorphism, nuclear hyperchromatism, nuclear enlargement, and bizarrely-shaped nuclei.
Term
Direct extension is a common mode of spread in OSCC (true/false).
Definition
True
Term
What are two types of carcinomas that are prone to seeding in the peritoneal cavity?
Definition
Colon and ovarian carcinoma
Term
Sarcomas normally spread via _____________; these two organs are the common endpoints.
Definition
Hematogenous spread affecting the liver and lungs; veins are the common route and arteries are rare.

Carcinomas are more likely to spread in the lymphatics.
Term
A tumor is first palpable once it contains ____ number of cells.
Definition
10^9 cells weighing 1 gram.
Term
T staging for a primary tumor
Definition
T0- no evidence of primary tumor
Tis- carcinoma in situ
T1- tumor <2cm
T2- tumor <4cm
T3- tumor >6cm
T4 (lip or oral cavity)- invasion of adjacent structures
Term
N staging for tumors
Definition
N0- single ipsilateral lymph node
N1- " " <3cm
N2a- " " <6cm
N2b- multiple ipsilateral lymph nodes, but NONE >6cm
N2c- bilateral, contralateral lymph nodes <6cm
N3- any lymph node >6cm!
Term
If you have an N1, the lowest stage you can get is Stage __.
Definition
Stage III (only if T1 or T2); can have Stage II with T3 as long as there's no lymph node involvement.
Term
Melanoma has been associated with the gene __.
Definition
p16
Term
Tumors have self-sufficiency in growth signals such as ____ in glioblastomas and ____ in sarcomas.
Definition
PDGF.....TGF-alpha

TGF-beta is a soluble growth inhibitor, so cancer cells knock this gene out as well.
Term
Bcl-2
Definition
Helps B-cells evade apoptosis in lymphomas.
Term
Cancer cells can avoid replicative senescence by mutating ____ or ___; they can avoid crisis by activating _____.
Definition
p53 or Rb.....activate telomerase
Term
Rho/Rac and MMPs aid in cancer's ability to.....
Definition
Have invasive potential
Term
Cachexia may be caused by.....
Definition
A cytokine elaborated by the tumor or the host.
Term
The telogen hypothesis proved that.....
Definition
The carcinogen needs to stay in contact with the area; we can't just wipe on and wipe off; latency would get in the way.
Term
Painting a second initiator on a newly formed papilloma can induce a carinoma (true/false).
Definition
True
Term
Genotoxic agents may be activated by __________ and antagonized by ____________.
Definition
Activated by CYP450 (a few are activated by Phase II metabolism)...........antagonized by reducing agents like glutathione, Vitamin C, and Vitamin E.
Term
Non-genotoxic carcinogens are generally good ___________.
Definition
Promoters
Term
Radiation causes these diseases....
Definition
1) Leukemia- most common
2) Thyroid cancer
3) Glands, lungs, etc.
Term
Radiation causes these effects on DNA.....
Definition
1) Cyclobutyl dimer (T-T dimers)- xeroderma pigmentosum
2) Deamination of cytosine producing uracil
3) Deletion of purinic sites- completely getting rid of adenine and a nucleotide pair on the new DNA
Term
RecA gene
Definition
Needed by cells to recognize unmethylated new DNA strands and perform mismatch repair on DNA damaged by UV radiation; here, the repair process actually causes the mutation (not the UV radiation).
Term
Ataxia telangectasia
Definition
Autosomal recessive syndrome of defective DNA repair caused by gamma radiation; affects genes that are involved in the signalling pathway to let cells know that DNA had been damaged and needs to be repaired.

Caused by mutations in ATM and hMre11 (less marked, later onset) genes.
Term
Fanconi's anemia is an autosomal recessive syndrome of defective DNA repair that interacts with the _________ gene.
Definition
BRCA1

Ubiquinated protein D moves to the nuclear foci that contain BRCA1.
Term
Bloom's Syndrome
Definition
Autosomal recessive syndrome of defective DNA repair that involves defects in a series proteins (RecQ subset of DexH box containing DNA helicases -- unwinding DNA).

Patients have short stature, sun-sensitive facial lesions, increased susceptibility to infections, infertility, and mental retardation.

Ashkenazi Jews on chromosome 15
Term
Cockayne Syndrome
Definition
Another autosomal recessive syndrome of defective DNA repair; these people are not susceptible to skin cancer although UV radiation causes CSA and CSB to no longer perform "transcriptional repair".
Term
Radium can cause ______ and ______ cancer while uranium can cause ______ cancer.
Definition
Bone and nose cancer.........lung cancer.
Term
If the oncogene from a transducing virus replaces gag, pol, or env, ____________________ is necessary.
Definition
Co-infection with a wild-type (helper) virus.
Term
Avian Leukosis Virus
Definition
Occurs in nature, but relies on "insertional mutagenesis"; can't transform a cell by itself like the Rous Sarcoma Virus.

May cause disease by activating c-myc from either end.
Term
__________ and _________ viruses are linked to a variety of animal tumors.
Definition
Polyoma virus and SV40
Term
Hepatitis B and C can cause hepatomas by......
Definition
Acting as mitogens or perhaps by scrambling chromosomes.
Term
HTLV-I causes ____________ while HTLV-II causes _____________.
Definition
Epidemic leukemia in Japanese............hairy cell "leukemia"
Term
Transforming proteins
Definition
Maintain the transformed state of a cell and are encoded by oncogenes.
Term
What phenotype would be expected from a mutation in hMSH or hMLH?
Definition
Hereditary NonPolyposis Colon Cancer (HNPCC), Lynch syndrome II
Term
What would be the phenotype expected in mutations in uvrABCD genes?
Definition
Xeroderma pigmentosum
Term
tat genes
Definition
A genome that contains tat genes in addition to gag, pol, and env are tumorigenic; associated with HTLV viruses.
Term
EBV binds......
Definition
C3 receptors on B cells; found in 100% of nasopharyngeal carcinomas! (8 to 14 translocation)
Term
HPV type __, __, and __ are associated with SCC in patients with epidermodysplasia verruciformis.
Definition
5, 8, and 14
Term
Oncogenes show dominant transmission; only one aberrant copy is sufficient for mutation (true/false).
Definition
True
Term
"ras" gene
Definition
Codes for GTP-binding proteins (like p21); this causes cancer by point MUTATION at codon 12 or 61 which makes the GTP-bound state more persistent (p21 is mutated in 30% of all human cancers).

Harvey-Ras associated with bladder cancer and N-Ras associated with colon and pancreatic cancer.
Term
Philadelphia chromosome
Definition
abl (Chrom 9) translocates with bcr (Chrom 22); activates tyrosine kinase and causes CML.

Shorter break points in the translocation cause ALL though.
Term
What was the first virus to be associated with human cancer?
Definition
EBV
Term
Homogenous staining regions and double minutes are associated with......
Definition
N-myc and neuroblastomas; HER-2/neu is a growth factor receptor that has a poor prognosis when amplified.
Term
____ and ____ are growth factor proteins that are actually ligands.
Definition
sis and hst

V-sis codes for a protein very similar to PDGF (normally C-sis produces true PDGF).
Term
______________ is overexpressed in stomach cancer.
Definition
Fibroblast growth factor (FGF)
Term
_____________ and _____________ produce PDGF, whereas normal cells do not.
Definition
Glioblastomas and sarcomas
Term
Gleevec
Definition
An anti-cancer drug that prevents CML by binding where ATP normally would.
Term
____ and ____ genes are nuclear regulatory proteins, and they need......
Definition
C-myc and c-fas take the cell out of Go phase; need PDGF stimulation to undergo mitosis and then need EGF or IGF to take the cell through S-phase.
Term
TP53
Definition
Located on chromosome 17 and is the MOST COMMON genetic defect found in cancers; induced in response to DNA damage and prevents the cell from entering S-phase.
Term
Mutations in the tumor suppressor gene DCC causes ____________.
Definition
Carcinomas of the colon and stomach.

Only one of multiple gene steps to cause colon cancer: need APC gene, K-ras, and possibly p53 as well.
Term
Wilm's Tumor Gene
Definition
A tumor suppressor gene; mutations linked to childhood renal cancer.
Term
EWS
Definition
A putative tumor suppressor gene expressed ubiquitously in all tissues; mutations linked to Ewing's sarcoma, etc.
Term
Mutations in Bcl-2 are different that other proto-oncogenes because......
Definition
Overexpression of Bcl-2 allows the cell to evade apoptosis.
Term
What other genes predispose women to breast cancer when mutated besides BRCA1 and BRCA2?
Definition
1) p53 (Li-Fraumeni Syndrome)
2) CD1 (Cowden Syndrome)
3) ATM (ataxia telangectasia mutated)
Term
________ is an oncogene that when activated causes a hereditary cancer.
Definition
RET causing MEN 2
Term
What qualifications does a cell need to cause oral cancer?
Definition
1) eliminate Rb (heterozygous loss which normally inhibits E2F)
2) Disable p53
3) Activate telomerase (made up of proteins and RNA)
4) Acquire a growth-promoting mutation
Term
How is the APC tumor suppressor gene regulated?
Definition
Normally, beta-catenin binds APC forming a "destruction complex"; when WNT binds to the cell, beta-catenin can translocate to the nucleus where it binds to TCF.

Causes Familial Adenomatous Polyposis
Term
Lynch Syndrome
Definition
HNPCC; usually occurs on the RIGHT side of the colon; 50% chance of passing gene on to offspring; individual has an 80% chance of developing a malignant polyp at some point in life.

Controlled by the genes MSH2 and MLH1.
Term
Calcium susceptibility syndrome may affect (gatekeeper/caretaker) genes.
Definition
Gatekeeper genes
Term
Mutations in caretaker genes eventually results in.......
Definition
Genomic instability leading to downstream mutations on gatekeeper genes.

Genomic instability is a big part of oral cancer.
Term
The patched gene is associated with which type of cancer?
Definition
Basal cell nevus syndrome
Term
Gardner Syndrome
Definition
A variant of familial adenomatous polyposis (both on Chromosome 5); an autosomal dominant mutation that causes osteomas of the skull, mandible, and long bones!
Term
__________ is associated with a rare type of male breast cancer.
Definition
BRCA2

Women with a mutation in BRCA1 or BRCA2 have up to a 90% chance of developing breast cancer and a 60% chance of developing ovarian cancer.
Term
BRCA genes
Definition
Are tumor suppressor genes located on chromosome 17(BRCA1) and 13(BRCA2)!
Term
Loss of Heterozygosity
Definition
Loss of normal function of one allele of a gene in which the other allele was already inactivated; requires a two-step process.

Wilm's tumor is an example where it is always the allele on the maternal chromosome 11 that is lost.
Term
Why is chromosome 9p important in the development of oral cancer?
Definition
Its mutation leads to p16 inactivation and hyperplasia (the first step to cancer).

Chromosome 3p goes next causing dysplasia along with p53 on chromosome 17.
Term
Do mutations in gatekeeper or caretaker genes have a greater effect on the development of oral cancer?
Definition
Caretaker genes

TP53 (Li Fraumeni Syndrome) is the only gatekeeper gene that predisposes to oral cancer.
Term
_____ inactivation leads to carcinoma in situ finally becoming invasive.
Definition
pTEN
Term
OSCC is more likely to present as a (leukoplakia/erythroplakia).
Definition
Erythroplakia
Term
Leukoedema is marked by intraepithelial edema in the ________ layer.
Definition
Spinous
Term
Fordyce granules
Definition
Ectopic sebaceous glands usually found in older individuals.
Term
Geographic tongue has this histological appearance.
Definition
Spongiosis and munro abscesses (PMNs in epithelium).

Looks identical to psoriasis in the skin.
Term
___________ is the one epithelial lesion that probably doesn't warrant biopsy.
Definition
Linea alba; a variant of focal (frictional) hyperkeratosis
Term
Which leukoplakia has no risk for transformation into cancer?
Definition
Hairy leukoplakia
Term
In papillomas, E6 causes ___________ while E7 causes _____________.
Definition
p53 inactivation............Rb inactivation

Koilocytic changes are characteristically seen in HPV-associated lesions.
Term
Condyloma acuminatum differs from papillomas by.......
Definition
They are more broad-based and exclusively transmitted sexually.
Term
White sponge nevus
Definition
Mutations in keratin 4 or 13 causing parakeratosis, epithelial acanthosis, and spongiosis.

A perinuclear eosinophilic halo is pathognomic!
Term
Hereditary Benign Intraepithelial Dyskeratosis (HBID)
Definition
Soft, asymptomatic white plaques; also forms foamy, gelatinous plaques of conjunctiva; epithelial acanthosis and edema with characteristic dyskeratotic cells in superior half of epithelium.
Term
Pachyonychia congenita
Definition
Mutations in keratin 6, 16, or 17 usually involving the tongue; skin and nail changes are often seen.
Term
Dyskeratosis congenita
Definition
Only inherited condition that can be autosomal dominant, recessive, or X-linked; mutations in telomerase gene causing skin and nail changes as well as affecting the tongue and buccal mucosa.

Risk to OSCC is increased.
Term
__% of erythroplakias are OSCC.
Definition
50%; much more than the 3% of leukoplakias.
Term
For which type of lesion do we need to biopsy a piece of the adjacent tissue?
Definition
Ulceration
Term
Pigmented lesions are always pathologic (true/false).
Definition
True
Term
Smoking-Associated Melanosis
Definition
Cigarette smoke increases melanocyte activity particularly in anterior labial gingiva; females on birth control are more susceptible.
Term
Vascular lesions are pigmented (true/false).
Definition
False
Term
Oral Melanotic Macule
Definition
Unknown origin but may represent post-inflammatory traumatically induced pigmentations.

Melanin incontinence (melanin in the connective tissue) may be seen.
Term
Systemic diseases associated with pigmentation.....
Definition
1) Peutz/Jaghers syndrome (susceptible to intestinal polyposis)
2) McCune-Albright's syndrome
3) Neurofibromatosis
4) Addison's disease
5) Cushing's syndrome
Term
Progession of a nevus....
Definition
1) Junctional nevus
2) Compound nevus
3) Intramucosal nevus

Oral nevi present as flat or elevated, and are frequently non-pigmented.
Term
The three most common forms of skin cancer are.....
Definition
1) BCC
2) SCC
3) Melanoma
Term
What is the most common pigmented lesion of the oral mucosa?
Definition
Amalgam tattoo
Term
Addison's disease
Definition
Hypoadrenalcorticism; recognized by increased ACTH levels; melanocyte-stimulating hormone (MSH) is part of the ACTH.
Term
Cushing's syndrome
Definition
Hyperadrenalcorticism; most causes are iatrogenic; only the ones that involve the pituitary gland cause pigmentation.
Term
Warts present as.....
Definition
Finger-like projections with pinpoint bleeding; there is a thickened granular cell layer.
Term
Histology of herpes simplex and varicella zoster viruses.....
Definition
Intraepidermal blister with acantholysis and multi-nucleated giant cells; nuclei have chromatic border with pale centers.
Term
Molluscum Contagiosum
Definition
Caused by the pox virus; small discrete skin-colored or pink papules with a central dell; endophytic epidermis with magenta-colored molluscum bodies.
Term
Tinea is caused by __________.
Definition
Trichophyton sp. (a dermatophyte, not yeast).

KOH stain shows the hyphae.
Term
Histology of tinea......
Definition
Epidermal thickening with neutrophils in the stratum corneum!
Term
North American Blastomycosis
Definition
Deep fungal infection caused by blastomyces dermatitidis; has primary cutaneous, pulmonary, and systemic forms.

Shows pseudoepitheliomatous hyperplasia and granulomatous inflammation (giant cells have a fungal component).
Term
Differential diagnosis for Pseudoepitheliomatous hyperplasia....
Definition
1) Deep fungal infections
2) Halogenodermas (iodides, bromides)
3) Granular cell tumor (Schwannomas)
4) SCC
Term
Histology of impetigo....
Definition
Subcorneal neutrophils; below the BM.
Term
Histology of leprosy.....
Definition
Granulomatous inflammation (as opposed to epidermal thickening in tinea); mycobacterium leprae present as well.
Term
Secondary syphilis is marked by......
Definition
Lichenoid (WBCs at the dermal/epidermal junction) and perivascular inflammation; plasma cells and Warthin Starry stain.
Term
Seborrheic keratosis
Definition
Extremely common "barnacles" stuck on the skin that are often pigmented.

Histologically see a pseudohorn cyst filled with keratin.
Term
Epithelial nevus
Definition
Looks like little papules in an arched line; histologically looks like seborrheic keratosis.
Term
Nevus Sebaceus of Jadassohn
Definition
Yellow hairless plaque on face or scalp present at birth; histologically see epidermal thickening with aborted hair follicle.

Tumors can develop in this lesion!
Term
BCC, nodular type
Definition
Most common! See telengiectasia; the head and neck are more commonly affected than the trunk and extremities.
Term
BCC, superficial type
Definition
Pink patch; second most common; trunk and extremities are more commonly affected than the head and neck.
Term
Histology of BCC......
Definition
Peripheral palisading and retraction artifact (characteristic).
Term
Which two forms of BCC have a high rate of recurrence?
Definition
Micronodular and morpheaform
Term
SCC has a ________ surface when on the skin.
Definition
Crusted
Term
Keratoacanthoma
Definition
Clinically resembles SCC, but looks like a volcano histologically; treated the same as SCC.
Term
Levels of melanoma
Definition
1) in situ (Lentigo Maligna- due to chronic sun exposure)
2) in the papillary dermis
3) filling the papillary dermis
4) into the reticular dermis
5) into the subcutaneous fat
Term
Koebner's phenomenon
Definition
Non-specific trauma can lead to formation of psoriasis in the area of irritation
Term
Histology of psoriasis
Definition
Epidermal thickening (acanthosis), parakeratosis with PMNs, and loss of granular cell layer.
Term
Seborrheic Dermatitis
Definition
Erythematous scaly "greasy" patches with a "Seborrheic distribution"; more severe in HIV patients; may be caused by Pityrosporum.
Term
Histology of lupus
Definition
Vacuolar alteration with lymphocytes at dermal-epidermal junction; dermal mucinous ground substance.
Term
Vitiligo
Definition
Depigmented area of skin; absence of melanocytes.
Term
Ranula
Definition
Mucocele on the floor of the mouth; not a true cyst: surrounded by granulation tissue.
Term
Salivary duct cyst
Definition
A true cyst that occurs commonly on the palate due to salivary gland obstruction.
Term
Sialadenitis
Definition
Inflammation of the major or minor salivary glands; atrophy of the glands and ductal dilation; may also see fibrosis.

There is no fever in chronic sialadenitis!
Term
Type of cheilitis glandularis
Definition
1) Simple- red, tender
2) Superficial suppurative (Baelz's disease)
3) Deep suppurative

Only superficial suppurative and deep suppurative are infectious.
Term
Cat-Scratch Disease
Definition
Caused by Bartonella henselae; the gland itself is not affected; we see necrotizing lymphadenitis (liquefactive); use Warthin-Starry silver stain to identify the bacillus; erythromycin (first choice) and doxycyclin can treat it.
Term
Mumps
Definition
Caused by a paramyxovirus infection; contagious until 14 days after clinical resolution; causes epididymoorchitis in males and spontaneous abortion in females who become pregnant during 1st trimester.
Term
Cytomegalic sialadenitis
Definition
Biopsy shows "owl eye" cells (pathognomic).
Term
Heerfordt's syndrome
Definition
Parotid enlargement, facial nerve palsy, uveitis (in eye), and fever associated with sarcoidosis.
Term
Sialadenosis
Definition
Non-neoplastic, non-inflammatory enlargement of salivary glands; usually caused by underlying systemic diseases.

Diminished salivary secretions with increased K and decreased Na.
Term
Sjogren's Syndrome
Definition
Shows no evidence of fibrosis or destruction of architecture; Schirmer test identifies ocular involvement; anti-nuclear antibodies (anti-SS-A and anti-SS-B).
Term
Benign Lymphoepithelial Lesion
Definition
Develops as a component of Sjogren's; dense infiltration of glands with lymphoid cells (parotid); surrounding myoepithelial cells become hyperplastic forming epimyoepithelial islands.

Increased risk for lymphoma.
Term
Pleomorphic Adenoma
Definition
Mutations in PLAG1 (tumor suppressor gene); forms a benign, encapsulated tumor of DOUBLE-LAYER ducts, plasmacytoid cells, and chondromyxoid tissue.

Warthin's tumors- only in major glands.
Term
Monomorphic Adenoma
Definition
Most frequent site is the upper lip; canalicular adenoma is a variant intraorally; can get severely deforming!
Term
Oncocytoma and myoepithelioma
Definition
Tumor made up of mainly mitochondria-rich tumor cells.........resembles pleomorphic adenoma except there are no double-layer ducts.
Term
Mucoepidermoid Carcinoma
Definition
Most common malignant neoplasm in salivary glands; affects the palate and parotid glands the most; tumor produce mucus as well as contain squamous cells.

Low-grade=good prognosis High-grade=poor prognosis
Term
Adenoid Cystic Carcinoma
Definition
Affects all salivary glands equally, but the palate is the most common site intraorally; has a classic histologic "Swiss cheese" appearance; deceptively good 5-year prognosis with dismal 15-year prognosis.
Term
Polymorphous low-grade adenocarcinoma
Definition
Exclusively affects minor salivary glands
Term
Acinic cell carcinoma
Definition
Second most common salivary glands malignancy in children (behind mucoepidermal carcinoma).
Term
Carcinoma ex mixed tumor
Definition
Pleomorphic adenoma that has become malignant.
Term
____________ are the most common oral soft tissue growths.
Definition
Fibromas

Giant cell fibroma has large, stellate-shaped fibroblasts with multi-nucleated cells.
Term
Neurofibromatosis type I (von Recklinghausen's disease)
Definition
Caused by mutation in the tumor suppressor gene neurofibromin on chromosome 17; no significant sex predilection, and half of all cases are not associated with a family history.

Oral manifestations are common with half of all patients experiencing enlargement of the FUNGIFORM papillae of the tongue.
Term
Diagnosis for NF1
Definition
1) Six or more cafe au lait
2) Two or more neurofibromas or one plexiform neurofibroma
3) Crowe's sign (freckling in the inguinal or axillary regions)
4) Optic glioma
5) Two or more Lisch nodules (iris hamartomas)
6) A first-degree relative with NF1
Term
Schwannoma
Definition
Caused by mutations in merlin tumor suppressor gene on chromosome 22 (associated with NF2); usually develop of solitary, non-pigmented lesions that are encapsulated and contain Antoni type A and B tissues as well as Verocay bodies.

Unlike neurofibromas, true nerve tissue is not found within these tumors.
Term
Mucosal neuroma
Definition
Associated with MEN-2b; caused by mutations in the RET proto-oncogene; also causes cancers of the thyroid and adrenal glands.

Usually presents as an unencapsulated mass of irregular, convoluted nerve bundles within normal connective tissue.
Term
Pathognomonic evidence of mucosal neuromas
Definition
Affects ocular and GI mucosa (GI bleeds); oral mucosa is almost always the first to be affected (mainly lips and tongue); high-arched palate and malocclusion.

BILATERAL NEUROMAS ON THE COMMISSURES!
Term
Hemangiomas are more common in (boys/girls).
Definition
Girls

Capillary hemangioma is the most common form.
Term
Sturge-Weber Angiomatosis
Definition
Non-hereditary defect of the vascular plexus around the cephalic portion of the neural tube; follows branches of the trigeminal nerve (port-wine stain or nevus flammeus).

Unique "tramline" calcifications of the ipsilateral brain!
Term
Lymphangioma
Definition
Benign vascular lesions of lymphatic vessels; occur early in life; cavernous version is the most common; affects anterior 2/3 of tongue and posterior cervical triangle more frequently; cystic hygromas seen.

Recurrence is common.
Term
Histology of angiomyomas (variant of leiomyoma)
Definition
Well-delineated nodules consisting of fascicles of smooth muscle cells surrounding thick-walled vascular channels.

Leiomyomas have cigar-shaped or blunt-ended nuclei.
Term
Granular cell tumor
Definition
Benign lesion that may be of neural origin; twice as common in women; poorly circumscribed infiltration of neoplastic cells; histologically see polygonal cells with pale, eosinophilic, granular cytoplasm; half of cases are associated with pseudoepitheliomatous hyperplasia.

Histologically, looks almost identical to the congenital epulis.
Term
S100 protein
Definition
Indicates Schwann cell origin and helps to differentiate granular cell tumors from congenital epulis.

Also, congenital epulis NEVER shows pseudoepitheliomatous hyperplasia!
Term
Neurofibromas
Definition
Ill-defined, unencapsulated mass that exhibits fascicles of spindle cells with wavy or comma-shaped nuclei; occurs most commonly on the tongue intraorally.
Term
Congenital epulis
Definition
Occur almost always in female newborns; poorly circumscribed lesions that often show infiltration into surrounding tissues.
Term
Choritomas are frequently found.......
Definition
In the posterior 1/3 of the tongue near the foramen cecum (80%) with cartilage and/or bone.
Term
Kaposi's sarcoma
Definition
Classic form, endemic form, and AIDS-related form; not caused by translocations but by HHV8! Resembles hemangioma, pyogenic granuloma, and ecchymosis; has decreased due to HAART therapy.
Term
Rhabdomyomas occur more frequently than rhabdomyosarcomas (true/false).
Definition
False
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