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
|
|
Term
signs of von hippel lindau disease |
|
Definition
hemangioblastoma of cerebellar hemisphere, retina, and sometimes brainstem and spinral colrd
cyst of pancreas, liver, kidney
high risk of renal cell carcinoma |
|
|
Term
characteristics of the lesion in von hippel lindau disease |
|
Definition
highly vascular neoplasm, large fluid cyst, PAS positive, lipid rich cytoplasm |
|
|
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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