Buzz Words Flashcards

1
Q

1p19q deletion

A

Oligodendroglioma, better prognosis

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2
Q

IDH1 mutation

A

Low grade glioma

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3
Q

Treatment for glioblastoma

A

Surgical resection/debulking
Radiation
Temozolomide (alkylating agent)

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4
Q

P53 mutation

A

Anaplastic Astrocytoma
Mutation present in 50%
Grade iii , survival 2-3 years

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5
Q

Well demarcated tumor in temporal lobe (cortical) , causes focal epilepsy

A

PXA. Resectable, good prognosis but 15-20% become malignant

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6
Q

“Candle gutterings”

A

Masses around vents associated with SEGAs (TSC and young people)

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7
Q

Infiltrating tumor in frontal cortex of a middle aged person

A

Oligodendroglioma

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8
Q

Paraneoplastic syndrome: retinal degradation (antibody, tumor?)

A

Anti-recoverin (anti-CAR)
Small cell lung, renal cell, melanoma, thymoma

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9
Q

ganglioglioma

A

neurons+glia, temporal lobe of kids and adults, causes seizures. MRI shows cyst w/ mural nodule. good prognosis, Grade I, just cut it out. path shows eosinophilic granular bodies

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10
Q

myxopapillary variant

A

a variant of ependymoma that arises from filum terminale, seen in adults, good prognosis (Grade I)

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11
Q

ependymoma genetics

A

assoc with 22q deletions and NF2

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12
Q

what 2 things are assoc with worse prognosis for ependymoma?

A

age of presentation younger than 3 years
drop mets

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13
Q

sheets of cells and perivascular pseudorosettes on path

A

ependymoma

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14
Q

ependymoma

A

3rd most common tumor in kids
infratentorial usually
perivascular pseudorosettes, sheets of cells
drop mets and <3 - worse prog

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15
Q

meningioma

A

F>M, age 60-70
arise from arachnoid cap cells
dural based - dural tail on imaging
PSAMMOMA bodies
assoc with 22q del, NF2, prior radiation, breast cancer

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16
Q

psammoma bodies

A

meningioma

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17
Q

most common primary cerebellar tumor in adults

A

hemangioblastoma

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18
Q

hemangioblastoma presentation

A

cerebellar mass in adults.
mass effect, CSF obstruction –> increased ICP
secondary polycythemia in 10% (secrete erythropoetin like substance)
cyst with mural nodule on MRI
25% of cases assoc with Von Hippel Lindau

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19
Q

hypercellular, small blue cells, Homer Wright rosettes

A

medulloblastoma - invasive cerebellar tumor. 20% of kid tumors

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20
Q

pineal gland tumors (pineocytoma vs pineoblastoma)

A

p/w with ophtho stuff (parinaud), brainstem, cerebellar, incr ICP, hypothalamic dysfunction

cytomas –> young adults, well demarcated
blastomas –> poorly demarcated, can spread via craniospinal axis or metastasize. worse prognosis. assoc chrom 11q

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21
Q

cyst that forms near foramen of Monro, can cause headache and AMS, intermittent syncope with valsalva. if obstruction - patients can have drop attacks, hydrocephalus, death.

A

colloid cyst

T1 signal, small enhancing rim

22
Q

cyst that arises from ectopic ectodermal tissue, in young adults, from cerebellopontine angle, DWI diffusion restricting

A

epidermoid cyst

CSF intense on t2, restricted diffusion on DWI (distinguishes it from arachnoid cyst)

23
Q

cyst that arises in kids in cerebellar vermis/parasellar/parapontine region and in lumbosacral spinal cord. pearly white structure containing hair follicles, sebaceous glands, sweat glands

A

dermoid cyst

if ruptures –> chemical meningitis with granulomatous inflammation

24
Q

where are arachnoid cysts typically seen

A

temporal lobes, meninges

25
Q

where does Rathke’s cleft cyst occur

A

between anterior and posterior hypophysis

26
Q

embryonal tumor that arises from pluripotential cells, seen in kids, more common in boys, posterior fossa

A

medulloblastoma

incr ICP, hydrocephalus, cerebellar findings

27
Q

why should you get a spine MRI if you see medulloblastoma in cerebellum

A

can metastasize via CSF

28
Q

poor prognosis in medulloblastoma

A

<3 years at onset
incomplete resection
brainstem involvement
mets
N-myc transcription factor amplification

29
Q

refractory sz in kids - what tumor? grade?

A

DNET - cortical/juxtacortical, temporal lobe

WHO Grade I, resection curative

30
Q

primary CNS lymphoma

A

immunocompromised patients (HIV, transplant)
EBV association
B-cell lymphoma
can involve the eye - get ophtho exam
poor prognosis
tx: steroids, but after biopsy, radiation, chemo, HAART in HIV patients

31
Q

tumor causing visual disturbances, kids and middle aged adults

A

craniopharyngioma
multicystic, benign, arise from Rathke’s pouch
mass effect –> optic chiasm, pituitary, hypothalamus, vents
lipid and cholesterol rich fluid inside
tx surgical resection

32
Q

what is a chordoma and where do they occur

A

invasive bone-destructive tumor in adults (M>F)
located in clivus (skull base) and sacrococcygeal region

clivus –> HA, cranial neuropathies due to brainstem compression, neck pain

sacrococcygeal –> sphincter dysfunction, pain

33
Q

unilateral hearing loss, tinnitus, ataxia, circumscribed contrast enhancing tumor in cerebellopontine angle

A

vestibular schwannoma

34
Q

brain mets - most common source?

A
  1. lung
  2. breast
  3. melanoma

80% supratentorial.

very rare to have mets from prostate, esophagus, other skin cancers

35
Q

most common tumors of cerebellopontine angle

A

acoustic schwannoma
meningioma
epidermoid cyst (diffusion restricts, chemical meningitis)

36
Q

man with MGUS and peripheral neuropathy. work-up?

A

anti-MAG antibody - (myelin associated glycoprotein)

37
Q

paraneoplastic syndrome causing brainstem encephalitis, assoc with lung and breast cancer

A

Anti-Ri/ANNA-2

38
Q

kid with cerebellar tumor, cystic with mural nodule. dx and prognosis?

A

pilocytic astrocytoma
grade I , slow growth + good prognosis
surgical resection, +/- radiation if can’t completely resect
assoc with NF1 (usually optic pathway)

39
Q

oligodendroglioma vs central neurocytoma

A

both have fried eggs on histopath

oligos are +IDH mutation and +1p/9q codeletion

central neurocytomas - synaptophysin

40
Q

most common cancer assoc with opsoclonus myoclonus syndrome in adults?c

A

small cell lung cancer

if anti-Ri/ANNA-2 –> breast cancer (second most common cancer)

41
Q

pineal region tumor seen on MRI - next step?

A

check levels of AFP and b-HCG - if normal, proceed with biopsy.

resect mature teratomas only, not clear if this helps germ cell tumors

42
Q

pineoblastoma management

A

get MRI spine - can seed neuroaxis
check CSF for malignant cells
resection, brain/spine radiation, chemo

43
Q

anti-Hu (ANNA-1) symptoms

A

sensory predominant peripheral neuropathy
cerebellar ataxia
limbic encephalitis
sensory neuronopathy

most common in small cell lung cancer

44
Q

anti-LG1 symptoms

A

faciobrachial dystonic seizures
hyponatremia
memory probs
myoclonus

45
Q

anti-Yo antibody symptoms, cancer

A

breast and pelvic cancer

cerebellar degeneration –> ataxia

46
Q

most important prognostic biomarker for good prognosis in medulloblastoma?

A

WNT activation –> 100% 5 year survival
(will not tank)

47
Q

prognostic biomarker for poorest outcome in medulloblastoma?

A

MYC amplification
(May You Cherish….your time left)

48
Q

bubbly temporal mass in a kid with refractory seizures

A

DNET -

glioneural elements on path
resect it!

49
Q

IDH positive + 1p/19q codeletion

A

oligodendroglioma

50
Q

p53 + IDH mutation

A

astrocytomas

51
Q

girl with cranial nerve deficits and ataxia, hyperreflexia found to have large expansile brainstem mass. dx and tx?

A

DIPG. radiation only

52
Q

intracellular reticulin deposition

A

PXA