Term
| CNS/skull cancer: percent metastic, most common location, most common ages |
|
Definition
50/50 metastic and primary more often intracranial than spinal large portion of childhood cancers 20% |
|
|
Term
| where is a brain tumor most often located in a kid |
|
Definition
| posterior fossa or sellar region |
|
|
Term
| where is a brain tumor most often located in a adult |
|
Definition
|
|
Term
| what are the general symptoms of a supratentorial tumor |
|
Definition
| slow growing with generalized or focal seizures |
|
|
Term
| what are 6 differences in the histology and behavior of CNS tumors than other tumors |
|
Definition
no-premalignant in situ stage low grade lesion can infiltrate large regions anatomical site can influence outcome independent of grade due to local effects on the brain even high malignant gliomas rarley spread outside CNS suprarachnoid space allows for spread to distant sites and large regions of brain usually have low mitotic rate, cellular uniformity, slow growth |
|
|
Term
| what do the signs and symptoms of a brain tumor dependent on |
|
Definition
| size, location, rate of growth, surrounding cerebral edema |
|
|
Term
| general signs of a brain tumor 4 |
|
Definition
increased ICP: early morning headache and nausea, sunset sign
edema: symptoms dependent on location
generalized or focal seizure: supratentorial compartment tumors (not primairly grneralized at first they spread from local, primary is always inherited
sudden change in neurological status: hemorrhage into vascular tumor (often vascular meningiomas) |
|
|
Term
|
Definition
| drooping of eyelids in the morning |
|
|
Term
| what is the best why to look at a brain tumor, why |
|
Definition
MRI with and without contrast CT cannot see posterior fossa (most common kid tumor area)
contrast helps differentiate between tumor and surrounding edema |
|
|
Term
| what are the benign tumors of the skull 4 |
|
Definition
osteoma chondroma hemangioma dermoid/epidermoid |
|
|
Term
| what are the malignant tumors of the skull 7 |
|
Definition
SCC of sinus, temporal bone, nasopharyngeal carcinoma spread to skull
primary... chondrosarcoma chondroma osteosarcoma glumus jugulare tumor |
|
|
Term
|
Definition
location: paranasal sinuses, mandible associated with gardner syndrome (familial polyposis) |
|
|
Term
| chondroma: location, diagnosis 2 |
|
Definition
location: skull base, paranasal sinuses lytic lesions on CT stippled calcification |
|
|
Term
|
Definition
|
|
Term
| dermoid/epidermoid location, tx |
|
Definition
all 3 layers of the bone no tx |
|
|
Term
| malignancies that spread to the skull bone 3 |
|
Definition
| SCC or sinuses, temporal bone, nasopharyngeal carcinoma |
|
|
Term
| how to identify a osteosarcoma |
|
Definition
| expansible, sclerotic border, isointense to normal bone |
|
|
Term
| glumus jugulare tumors aka |
|
Definition
|
|
Term
| glumus jugulare tumors cause |
|
Definition
| come from chromaffin cells near jugular bulb and secrete catecholamines |
|
|
Term
| glumus jugulare presenting symptoms |
|
Definition
| labial HTN due to catecholamines |
|
|
Term
| what are the 2 nerve sheath tumors |
|
Definition
vestibular schwannoma (acustic neuroma) trigeminal schwannoma |
|
|
Term
| vestibular schwannoma: age, gender, signs 3 |
|
Definition
40-60yo women > men unilateral progressive hearing loss and tinnutis, unsteady gait |
|
|
Term
| vestibular schwannoma: locations 4 |
|
Definition
10% cranial tumors - very common 90% from vestibular branch of VII 10% from the cochlear branch of VII extend into autitory meatus |
|
|
Term
| vestibular schwannoma: diagnosis, tx |
|
Definition
stains for S-100 surgery if >3cm (because surgery damages nerves 50% of time) |
|
|
Term
| signs of trigeminal schwannoma 4 |
|
Definition
numbness, weakness, pain in trigeminal distribution growth leads to involvement of VII and VIII |
|
|
Term
| what are the most common tumors in the supratentorial hemisphere region 3 |
|
Definition
|
|
Term
| what are the most common tumorsin the supratentorial midline region 3 |
|
Definition
pituitary adenoma pineal craniphyaryngioma |
|
|
Term
| what are the most common intratentorial tumors in adults 4 |
|
Definition
acoustic schwannoma metastasis meningioma hemangioma |
|
|
Term
| what are the most common intratentorial tumors in kids 4 |
|
Definition
cerebellar astrocytoma medulloblastoma ependymoma brainstem glioma |
|
|
Term
| what is the most common tumor extradural spinal cord |
|
Definition
|
|
Term
| what is the most common tumors intradural spinal cord 4 |
|
Definition
extramedullary: meningioma, schwannoma neurofibromatoma
intramedulary: ependymoma, astrocytoma |
|
|
Term
| meningoma: age, fender, prevelance |
|
Definition
benign tumor of adult 60-70yo female > male (unless kid) 20% of all intracranial tumors |
|
|
Term
|
Definition
radiotherapy deletes 22q derived from arachnoid meningothelial cells blood supply from external carotid can remove blood supply and let it shrunk |
|
|
Term
|
Definition
| EMA (epithelial membrane antigen) positive |
|
|
Term
|
Definition
parasaggital free convexity falcine olfactory groove suprasellar foramen magnum (often with obstrictuve hydrocephalus) sphenoid ridge attached to dura in ventricles |
|
|
Term
| clinical signs of meningoma 1 |
|
Definition
| vauge non-localizing symptoms of brain compression |
|
|
Term
| what are the histological grades and names of of meningomas 4 |
|
Definition
grade 1-4: meningoma grade 2-4: atypical meningoma grade 3-4: anaplasmic/melignant meningoma multiple |
|
|
Term
| what is the biggest concern with a grade 1 meningoma |
|
Definition
|
|
Term
| what are the types of meningoma 5 |
|
Definition
syncytial fibroblastic transitional plasmmomatous secretory |
|
|
Term
| synctial meminboma: description |
|
Definition
| whorled clusters of cells without visible cell membrane in tight groups |
|
|
Term
| fibroblastic meningoma: description |
|
Definition
| elongatd cells, collagen deposition between |
|
|
Term
| transitional meningoma: description |
|
Definition
| synctial and fibroblastic |
|
|
Term
| psammomatous memingoma: description |
|
Definition
|
|
Term
| secretory meningoma: description |
|
Definition
| gland like PAS positive eosinophilic secretion (pseudopasmmoma bodies) secretes VEGF and causes edema |
|
|
Term
| atypical meningoma: describe nucleus, growth pattern and rate |
|
Definition
promonent nucleoli increased cellularity, high mitotic rate patternless growth agressive local growth higher rate of reoccurrance |
|
|
Term
|
Definition
| a cell of atypical meningoma which is more agressive |
|
|
Term
| malignant meningoma: behavior, complications |
|
Definition
highly agressive resemble high grade sarcoma or carcinoma systemic metastasis in 50% |
|
|
Term
| where do malignant meningoma usually metastasize to 3 |
|
Definition
|
|
Term
| what is a papillary meningoma |
|
Definition
| anaplastic/malignant meningoma subtype that is treated as malignant |
|
|
Term
| what are multiple meningomas associated with 2, what are they caused by |
|
Definition
CN8 schwannomas, glial tumors
NF2: loss of function mutation in long arm ch22 in common with meningomas with fibroplastic, transitional, plasmmomatous growth |
|
|
Term
| how are meningomas diagnosed 3 |
|
Definition
CT: isointense or hyperdense, enhancement strong, homogenous, surrounding hypertosis in 25%
MRI: isointense, hypointense, intense enhancement
psammma bodies: calcified areas |
|
|
Term
| tx meningomas: asymptomatic |
|
Definition
follow with imaging steroids for edema |
|
|
Term
| tx meningoma: disabled pt |
|
Definition
surgery steroids for edema |
|
|
Term
| tx meningoma: residual tumor after surgery or malignancy |
|
Definition
x-ray therapy steroids for edema |
|
|
Term
| tx meningoma: tx that wont work 3 |
|
Definition
hormones chemo prophylactic anti-convulsants |
|
|
Term
| what are the 8 types of gliomas |
|
Definition
brainstem glioma pilocystic astrocytoma fibrillary (diffuse) astrocytoma gliomatosis cerebri cerebellar astrocytoma oligodendroglioma ependymomas turcot syndrome |
|
|
Term
| how do most gliomas present |
|
Definition
| 80% of low grade gliomas present with isolated seizure and no focal deficits |
|
|
Term
| prognosis of most gliomas |
|
Definition
| all are infiltrative except pliocytic |
|
|
Term
| how does a gluoma look on a MRI |
|
Definition
low grade: non-enhancing, best on T2 flair, usually frontotemporal
CONTRAT UPTAKE (ENHANCING) typically indicates malignant transformation |
|
|
Term
| brainstem glioma: who gets it |
|
Definition
|
|
Term
| brainstem glioma: symptoms 3 |
|
Definition
|
|
Term
| brainstem glioma: diagnosis |
|
Definition
| MRI: diffuse infiltration and enlargement of pons and rostral medulla |
|
|
Term
| brainstem glioma: location |
|
Definition
|
|
Term
|
Definition
cannot be resected not sensitive to radiation of chemo prognosis poor if focal (more demarcated) it is low grade and there is good prognosis after surgery but this is rare |
|
|
Term
| pilocystic astrocytoma: grade, metastasis?, age, composition |
|
Definition
grade 1 benign cystic - enlargement causes symptoms kids and adolescents |
|
|
Term
| pilocystic astrocytoma: cause |
|
Definition
associated with NF1
point mutation of tandem duplication in serone theroning kinase (BARF) no mutation in IDH1/2! |
|
|
Term
| pilocystic astrocytoma: cell types, description, behavior |
|
Definition
low cellularity bipolar/piloid hair like processes with GFAP rosenthal fibers eosinophilic granular bodies microcysts necrosis and mitosis rare |
|
|
Term
| what is the most common astrocytoma |
|
Definition
|
|
Term
| what is the most common adult primary tumors |
|
Definition
40-60yo 80% fibrillary/diffuse astrocytoma |
|
|
Term
| where are fibrillary astrocytomas |
|
Definition
|
|
Term
| signs of fibrillary astrocytomas 3 |
|
Definition
seizure headache focal deficits |
|
|
Term
| grades and names of fibrillary astrocytomas |
|
Definition
grade 2: benign astrocytoma grade 3: anaplastic astrocytoma (malignant) grade 4: glioblastoma multiform (malignant, agressive) |
|
|
Term
| compare the nucleus, cellular look, and behavior of the fibrillary astrocytomas |
|
Definition
grade 2: differentiated, gray, fibrillary astrocyts with glial fibrillary acidic protein (GFAP), increased cellularity
grade 3: nuclear atypica, mitosis, malignant
grade 4: mitosis, endothelial proliferation (firm, white, hemorrhage) OR necrosis (soft, yellow, cystic) |
|
|
Term
| what causes a benign grade 2 astrocytoma |
|
Definition
| mutation in IDH1 and IDH2 (metabolic enzyme isocitrate DH) |
|
|
Term
| how can a benign grade 2 astrocytoma be diagnosed |
|
Definition
|
|
Term
| compare the treatment and prognosis of the three grades of fibrillary astrocytoma |
|
Definition
grade 2: survery and EXT. survival 5y
grade 3: surgery, chemo, EXT. survival 3-8y
grade 4: metastasize within CNS, surgery, chemo, XRT, survival 1y |
|
|
Term
| cause of glioblastoma multiform |
|
Definition
loss of function mutation in p53 and RB tumor supressor
gain of function mutation in oncobenic P13K |
|
|
Term
| gliomatosis cerebri: cause |
|
Definition
| diffuse infiltration of neoplastic glial cells through a hemisphere or entire brain (variant of gliablastoma multiforme) |
|
|
Term
| gliomatosis cerebri: diagnosis 2 |
|
Definition
biopsy: diffuse astrocytoma looking MRI: diffuse, non-enhancing lesion in white matter, possible diffuse edema |
|
|
Term
| gliomatosis cerebri: prognosis |
|
Definition
|
|
Term
| gliomatosis cerebri: treatment |
|
Definition
|
|
Term
| cerebellar astrocytoma: who gets it, prognosis |
|
Definition
5-10yo slow growing, surgical excision curative, reoccurrence is uncommom, FU with MRI to monitor for reoccurrence |
|
|
Term
| cerebellar astrocytoma: location 2 |
|
Definition
| vermis, cerebellar hemisphere |
|
|
Term
| cerebellar astrocytoma: describe the lesion 3 |
|
Definition
associated witha cyst neoplastic mass mural nodule |
|
|
Term
| cerebellar astrocytoma: symptoms 4 |
|
Definition
irritability vomiting enlarged head ataxia |
|
|
Term
| oliogendroma: age, location 3, signs 2 |
|
Definition
40-50yo cerebral hemisphere white matter: frontal, temporal
several years of neurological complaints, seizures |
|
|
Term
| oligodendroma: grades and names |
|
Definition
grade 2: oligodendroma
grade 3: anaplastic oligodendroma |
|
|
Term
| compare the cell look and behavior of the 2 grades of oligodendroma |
|
Definition
grade 2: gray, cyst, gelatenous, focal hemorrhage, sheets of regular cells, spherical nuclei, granular chromatin, ROUND FRIED EGGS, CALCIFICATIONS, anastamosing capillaries
grade 3: high cell densiry, nuclear anaplasia, mitotic, vascular proliferation |
|
|
Term
| comare the treatment and survival of oligodendroma 2 grades |
|
Definition
grade 2: resection, imaging, chemo/XRT if progressive. 8-16y to decades
grade 3: usually responsive to chemo, survival 5y (10 if 1p/19q deletion which is more responsive to chemo and radiation) |
|
|
Term
| ependymomas: locations 4 and prevelance, who is most likley to have each location, which has the best and worse prognosis |
|
Definition
come from ependymal cells lining ventricles 70% 4th ventricle 0-12yo solid mass on ventricle flood 20% lateral ventricle 10% cauda equina - adult, best prognosis posterior fossa - worst prognosis |
|
|
Term
| what is adult ependymoma associated with 2 |
|
Definition
|
|
Term
| what are the grades and names of the ependymomas |
|
Definition
grade 2: ependymoma grade 3: anaplastic ependymoma |
|
|
Term
| compare the cells of the grades of ependymomas: formation, behavior, differentiation |
|
Definition
BOTH are SOLID papillary mass (not diffuse)
grade 2: perivascular PSEUDOROSETTES/EPENDYMAL HOMER WRIGHT ROSETTES (resemble embryologic ependymal canal)
grade 3: high mitotic rate, increased cell density, necrosis, ventricular proliferation, less epidemyal differentiation |
|
|
Term
| compare the treatment and prognosis of the ependymomas |
|
Definition
grade 2: possible late stage CSF dissemination, gross total excision
grade 3: CSF dissemination, gross total excision, post-op XRT |
|
|
Term
| what type of ependymoma cannot be removed, why |
|
Definition
| 4th ventricle location (in kids mostly) because it is attached to floor of medulla |
|
|
Term
| which glioma has to have post-op radiation of entire neural axis, why |
|
Definition
| anaplastic ependymoma. because it is a timor in the ventricle cells and can seed through out the entire CNS and they commonly reoccur after surgery |
|
|
Term
|
Definition
| mutation in 5q21 gene for DNA mismatch repair |
|
|
Term
| turcot syndrome: location, signs |
|
Definition
| multiple colorectal tumors and CNS neuroepithelial tumors including ALL GRADES of astrocytomas, medulloblastomas, and ependymomas |
|
|
Term
| what are the three neuronal tumors |
|
Definition
central neuroma galgnioneuroma dysembryoplastic neuroepithelial tumor |
|
|
Term
| central neurocytoma: location, behavior, nuclei |
|
Definition
adjacent to lateral third ventricle low grade evenly spaced, round, uniform, islands of neutrophils |
|
|
Term
|
Definition
low grade, slow growing astrocytoma glial part and become anaplastic |
|
|
Term
| ganglioneuroma: look of cells, symptoms |
|
Definition
seizures mature looking neurons mix of glial elements |
|
|
Term
| dysembryoplastic neuroepithelial tumors: location, look of neurons, symptoms, behavior, prognosis |
|
Definition
superificial temporal lobe FLOATING neurons in myoxoid fluid often have seiures slow grwing, low grade good prognosis after resection |
|
|
Term
| medulloblastoma: age, behavior, prognosis |
|
Definition
20% of pediatric tumors (2-10yo) highly malignant 70% survive 5y due to radiosensitivity |
|
|
Term
| medulloblastoma: location in kids and adults |
|
Definition
midline in kids lateral in adults can extend to leptomeninges |
|
|
Term
|
Definition
MYC amplifications: poor outcome WNT signaling mutations: better SHH pathway mutations: role in tumorgenesis |
|
|
Term
| medulloblastoma: cellular origin and MoA |
|
Definition
come from multipotential cells in cerebellum vermis and spread to 4th ventricle metastasis throguh CSF to spinal cord, base of brain, cerebral hemisphere |
|
|
Term
| medulloblastoma: symptoms 3 |
|
Definition
ataxia hydrocephalis headache |
|
|
Term
| medulloblastoma: 2 cell types, general lesion characteristics |
|
Definition
cell circumscribed, gray, friable small blue cells homer wright rosent cells |
|
|
Term
| small blue cells: arangement, size, contents, behavior, origin |
|
Definition
very cellular with sheets of anaplastic cells small, round, little cytoplas, hyperchromatic nuclei, mitotic primitive (like progenitor cells of developing CNS), limited differentiation |
|
|
Term
| what are homer-wright rosette cells |
|
Definition
| focal areas of neuronal differentiation scattered in the area of primitive small blue cells in a medulloblastoma |
|
|
Term
| medulloblastoma: treatment 2 |
|
Definition
surgical: complete resection rare due to location, invasiveness, and metastasis
radiological: highly radiosensitive, RADIATE ENTIRE NEUROAXIS |
|
|
Term
| what is the difference between a medulloblastoma and primitive neuroectodermal tumor (PNET) |
|
Definition
| same appearace of medulloblastoma but in different location in the CNS |
|
|
Term
| what are the 5 pituitary tumors |
|
Definition
secretory null cell (non-secretory) pituitary carcinoma infindibuloma macroadenomas |
|
|
Term
| pituitary tumors general info: symptoms, benign?, age, cellularity |
|
Definition
often asymptomatic almost always benign 30-40s all have pleomorphism and frequent mitoses on path |
|
|
Term
| how are pituitary tumors classified 4 |
|
Definition
size: micro <1cm, macro >1cm endocrine function clinical findngs histology |
|
|
Term
| what is the least common pituitary tumor |
|
Definition
|
|
Term
| what does a secretory pituitary tumor secrete 5 |
|
Definition
prolactin sometimes: GH, ACTH, FSH, LH |
|
|
Term
| why is a null cell tumor a problem |
|
Definition
| although does not secrete pituitary hormones may be invasive into cavernous sinus, vessels, and bone |
|
|
Term
| which pituitary tumor is metastasis, what is its behavior |
|
Definition
| pituitary carcinoma: DISTANT metastasis, highly invasive, anaplastic |
|
|
Term
| what is the name of a posterior pituitary tumor, what kind of tumor is it |
|
Definition
infindibuloma varient of pliocystic astrocytoma |
|
|
Term
|
Definition
| present with panhypopituitarism if nl pituitary destoried |
|
|
Term
| signs of macroadenoma and cause of each 5 |
|
Definition
headache: stretching of dura/diaphragm sellae transversed by V1
visual field cuts: compression of chiasm BEGINS WITH SUPERIOR TEMPORAL QUADRANTS THEN INFERIOR
diplopia: lateral extension in cavernous sinus CN III most effected
rhinorrhea: due to erosion of base of skull
pituitary apoplexy: hemorrhage into pituitary |
|
|
Term
| what are the signs of pituitary apoplexy 6 |
|
Definition
sudden onset headache CN III palsy nausea/vomiting AMS diplopia rapidly progressive vision loss |
|
|
Term
| treatment of prolactinomas |
|
Definition
| correct endocrine malfunction via dopamine |
|
|
Term
| treatment of non-prolactoma secreting tumor |
|
Definition
|
|
Term
| treatment of asymptomatic pituitary tumor |
|
Definition
| macroadenomas should be followed with MRi and visual field tests |
|
|
Term
| treatment of refractory pituitary tumors |
|
Definition
|
|
Term
| no matter the pituitary tumor, after surgery the person should always..., why |
|
Definition
| get hormone checks because HYPOPITUITARISM CAN OCCUR FOR 610-10Y! |
|
|
Term
| what are the two types of CNS lymphoma |
|
Definition
|
|
Term
| location of metastic CNS lymphomas 3 |
|
Definition
10% leptomeninges 4% epidural space 1% brain |
|
|
Term
| when do metastic CNS lymphomas have high CNS involvement warrenting intrathecal chemo prophylaxis 5 |
|
Definition
older age marrrow or retroperitoneal involevement high LDH nasal sinus disease burkitt lymphoma |
|
|
Term
| signs of metastic CNS lymphoma |
|
Definition
CN III, VI, VII most effected CSF: B2 MICROGLOBULIN epidural: predilection for thoracic cord |
|
|
Term
| tx and prognosis of metastic lymphoma |
|
Definition
tx predilection for thoracic cord with CRT medial survival 4mo |
|
|
Term
| who gets primary CNS lymphomas 7 |
|
Definition
increasing in immunocompetent <60yo
increased in: AIDS, renal transplant, Wiscott-Aldrich, ataxia-telangectasia, IgA deficiency, RA |
|
|
Term
| what is onset of primary CNS lymphoma in an AiDS patient associated with |
|
Definition
|
|
Term
| pairmary CNS lymphoma: location 4, which is most common |
|
Definition
single or multiple parenchymal lesions in CNS ocular, leptomeninges, intradural cord, SUPRATENTORIAL (FRONTAL LOBE) IS MOST COMMON |
|
|
Term
| pairmary CNS lymphoma: what is the tumor made of, how is it triggered |
|
Definition
98% are DIFFUSE LARGE B CELL TUMORS TRIGGERED BY VIRUS (NOT CAUSED) |
|
|
Term
| pairmary CNS lymphoma: diagnosis 5 |
|
Definition
CT: isodense, hyperdense MRI: hypointense on T1, homogeneously enhances, poor demacrated borders
biopsy CSF vitreous fluid |
|
|
Term
| how do diagnostic signs of pairmary CNS lymphoma differe in an AIDS patient, what tests should you get |
|
Definition
AIDS imaging: RING ENHANCING LESION!! ANY HIV (OR SUSPECTEd) PT NEEDS LARGE BATTERY CSF TEST WHEN THEY HAVE HEARDCHES
PET, SPECT, CSF, EBV! |
|
|
Term
| what conditions have ring enhansing lesions5 |
|
Definition
| AIDS pairmary CNS lymphoma, TB, cryptococcus, toxoplasmosis, CNS abscess via staph |
|
|
Term
| pairmary CNS lymphoma: prognosis, response to tx |
|
Definition
agressive poor response to chemo 30-60 mo if tx survival with AIDS 4mo |
|
|
Term
| germ cell tumor: location, age, race, gender, name one and what does it look like, prognosis |
|
Definition
along midline: pineal in male and suprasellar 90% 0-20yo 10% of brain tumors in japanese male > female
germinoma: resembles testicular seminoma, benign brain germ cell tumor |
|
|
Term
| what percent of intracranial tumors are metastic |
|
Definition
|
|
Term
| if an intracranial tumor were to metastasize, where would it go |
|
Definition
| 80% breast, lung, skin (melanoma), kidney, GI |
|
|
Term
| histology og intracranial metastic tumor: demarcated, color, location, cells |
|
Definition
sharple demarcated masses at gray and white matter junction surrounded by zone of edema and reactive gliosis |
|
|
Term
| what are pareneoplastic syndromes associated with |
|
Definition
| small cell carcinoma of the lung |
|
|
Term
| what are 4 histological presentations of paraneoplastic syndromes |
|
Definition
subacute cerebellar degeneration
limbic encephalitis
subacute sensory neurpopathy
ovarian teratoma and andibodies against N-methyl-D-aspartate receptors |
|
|
Term
| signs of subacute cerebellar degeneration 4 |
|
Definition
ataxia destruction of purkinje cells gliosis inflammatory |
|
|
Term
| sign of limbic encephalitis |
|
Definition
|
|
Term
| signs of subacute sensory neuroptathy 2 |
|
Definition
altered pain sensation loss of neurons in dorsal root ganglia |
|
|
Term
| signs of ovarian teratoma and antibodies against N-methyl-D-aspartate receptors |
|
Definition
| syndrome of rapid onset psychosis, catatonia, epilepsy, coma |
|
|
Term
| what are the 2 familail tumor syndromes and their inheritence |
|
Definition
tuberous sclerosis von hippel lindau disease both dominant |
|
|
Term
| cause of tuberous sclerosis |
|
Definition
defect on ch19q and 16p causes loss of tumor supressor genes TSC1 (hamartin) and TSC2 (tuberin) which form dimer that regulates mTOR (senses cell nutrient status and regulates metabolism)
loss of protein causes increas in mTOR and increase in cell growth |
|
|
Term
| signs of tuberous sclerosis 7 |
|
Definition
hydrocephalus harmagomas benign neoplasms cysts of liver, kidney, pancreas cutaneous lesions seizures |
|
|
Term
| why is there hydrocephalus in tuberous sclerosis |
|
Definition
| proximity of harmatomas to foramen of monro |
|
|
Term
| what are the 4 harmatomas in tuberous sclerosis |
|
Definition
cortical tubers supependymal harmagoma subependymal giant cell astrocytoma retinal glial harmatoma |
|
|
Term
| why are there seizures in tuberous sclerosis |
|
Definition
| cortical tubers location dysrupt electrical activity |
|
|
Term
| 4 benign neoplasms associated with tuberous sclerosis |
|
Definition
ependymoma renal angiomyolipoma pulmonary lesions cardiac rhabdomyomas |
|
|
Term
| what are the 3 cutaneous lesions of tuberous sclerosis |
|
Definition
angioibromas: leathery thickning in localized shagreen patches hypopigmented areas: ash leaf patches subungal fibromas |
|
|
Term
| cause of von hippel lindau disease |
|
Definition
tumor supressor VHL encodes part of ubiqutin ligase that targets TF hypodix inducible factor (HIF) for degredation
loss of VHL function increases HIF and thus VEGF, GF, erythropoetin causing paraneoplastic polycythemia
mutation in tumor supressor gene VHL which controls angiogenessis (esp in response to hypoxia) |
|
|
Term
| what condition is associated with von hippel lindau disease |
|
Definition
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Term
| signs of von hippel lindau disease |
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Definition
hemangioblastoma of cerebellar hemisphere, retina, and sometimes brainstem and spinral colrd
cyst of pancreas, liver, kidney
high risk of renal cell carcinoma |
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Term
| characteristics of the lesion in von hippel lindau disease |
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Definition
| highly vascular neoplasm, large fluid cyst, PAS positive, lipid rich cytoplasm |
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Term
| what percent of cancer metastiasizes to the brain |
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Definition
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Term
| what are the 4 areas that metastasize most to the brain |
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Definition
lung colorectal renal melanoma |
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Term
| where does lung metastasis go in brain 2 and their symptoms |
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Definition
leptomeningeal: CN, spinal root, cerebrum
epidural: cord compression |
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Term
| where does breast metastasis go in the brain and their symptoms |
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Definition
breast>calvaria>dura (meningoma like, sclerotic inflammation)
breast> leptomeningeal (CN, spinal root, cerebrum, leptomeningeal carcinomatosis)
breast> epidural (cord compression) |
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Term
| what are the places with cancer that metastasize to brain 9 |
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Definition
lung breast lymphoma colorectal renal head/neck melanoma prostate hematologic |
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Term
| where does colorectal cancer go in brain and symptoms |
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Definition
| leptomeningeal (CN, spinal root, cerebrum) |
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Term
| where does melanoma go in brain, symptoms |
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Definition
| leptomeningeal (CN, spinal root, cerebrum) |
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Term
| where does prostate cancer go in brain and symptoms |
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Definition
prostate>calvaria> dura (meningoma like) prostate > epidural (cord compression) |
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Term
| where does hematologic cancer go in brain, symptoms |
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Definition
| leptomeningeal (CN, spinal root, cerebrum) |
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Term
| what are the most common cancers to cause brain hemorrhage 4 |
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Definition
melanoma renal cell carcinoma lung carcinoma CORIOCARCINOMA IN YOUNG WOMEN WHO JUST HAD MISSCARRIAGE |
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Term
| what are the most common cancers to metastasize to brain in kids (overall rare tho) 7 |
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Definition
germ cell tumor osteosarcoma neuroblastoma melanoma ewings sarcoma habdomyosarcoma wilms tumor |
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Term
| when cancer metastasizes to the epidural space, what percent is in what area causing compression |
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Definition
70% thoracic 20% lumbar 10% cervical |
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Term
| why is the metastic spread fo the thoracic area of the spine usually hematogenous, from what areas |
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Definition
it gets blood from two regions usually from lung, prostate, breast |
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Term
| symptoms of metastasis to epidural space 5 |
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Definition
pain aggravated laying down limb weakness autonomic disturbance sensory disturbance |
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Term
| diagnosis of metastasis to epidural space |
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Definition
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Term
| what cancers usually metastasize to epidural space, what causes the symptoms |
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Definition
symptoms caused by cord compression lung, breast, prostate |
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