Buzz Words

Question 1

1p19q deletion

Answer 1

Oligodendroglioma, better prognosis

Question 2

IDH1 mutation

Answer 2

Low grade glioma

Question 3

Treatment for glioblastoma

Answer 3

Surgical resection/debulking
Radiation
Temozolomide (alkylating agent)

Question 4

P53 mutation

Answer 4

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

Question 5

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

Answer 5

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

Question 6

“Candle gutterings”

Answer 6

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

Question 7

Infiltrating tumor in frontal cortex of a middle aged person

Answer 7

Oligodendroglioma

Question 8

Paraneoplastic syndrome: retinal degradation (antibody, tumor?)

Answer 8

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

Question 9

ganglioglioma

Answer 9

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

Question 10

myxopapillary variant

Answer 10

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

Question 11

ependymoma genetics

Answer 11

assoc with 22q deletions and NF2

Question 12

what 2 things are assoc with worse prognosis for ependymoma?

Answer 12

age of presentation younger than 3 years
drop mets

Question 13

sheets of cells and perivascular pseudorosettes on path

Answer 13

ependymoma

Question 14

ependymoma

Answer 14

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

Question 15

meningioma

Answer 15

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

Question 16

psammoma bodies

Answer 16

meningioma

Question 17

most common primary cerebellar tumor in adults

Answer 17

hemangioblastoma

Question 18

hemangioblastoma presentation

Answer 18

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

Question 19

hypercellular, small blue cells, Homer Wright rosettes

Answer 19

medulloblastoma - invasive cerebellar tumor. 20% of kid tumors

Question 20

pineal gland tumors (pineocytoma vs pineoblastoma)

Answer 20

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

Question 21

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.

Answer 21

colloid cyst

T1 signal, small enhancing rim

Question 22

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

Answer 22

epidermoid cyst

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

Question 23

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

Answer 23

dermoid cyst

if ruptures –> chemical meningitis with granulomatous inflammation

Question 24

where are arachnoid cysts typically seen

Answer 24

temporal lobes, meninges

Question 25

where does Rathke's cleft cyst occur

Answer 25

between anterior and posterior hypophysis

Question 26

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

Answer 26

medulloblastoma incr ICP, hydrocephalus, cerebellar findings

Question 27

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

Answer 27

can metastasize via CSF

Question 28

poor prognosis in medulloblastoma

Answer 28

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

Question 29

refractory sz in kids - what tumor? grade?

Answer 29

DNET - cortical/juxtacortical, temporal lobe WHO Grade I, resection curative

Question 30

primary CNS lymphoma

Answer 30

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

Question 31

tumor causing visual disturbances, kids and middle aged adults

Answer 31

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

Question 32

what is a chordoma and where do they occur

Answer 32

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

Question 33

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

Answer 33

vestibular schwannoma

Question 34

brain mets - most common source?

Answer 34

1. lung 2. breast 3. melanoma 80% supratentorial. very rare to have mets from prostate, esophagus, other skin cancers

Question 35

most common tumors of cerebellopontine angle

Answer 35

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

Question 36

man with MGUS and peripheral neuropathy. work-up?

Answer 36

anti-MAG antibody - (myelin associated glycoprotein)

Question 37

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

Answer 37

Anti-Ri/ANNA-2

Question 38

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

Answer 38

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

Question 39

oligodendroglioma vs central neurocytoma

Answer 39

both have fried eggs on histopath oligos are +IDH mutation and +1p/9q codeletion central neurocytomas - synaptophysin

Question 40

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

Answer 40

small cell lung cancer if anti-Ri/ANNA-2 --> breast cancer (second most common cancer)

Question 41

pineal region tumor seen on MRI - next step?

Answer 41

check levels of AFP and b-HCG - if normal, proceed with biopsy. resect mature teratomas only, not clear if this helps germ cell tumors

Question 42

pineoblastoma management

Answer 42

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

Question 43

anti-Hu (ANNA-1) symptoms

Answer 43

sensory predominant peripheral neuropathy cerebellar ataxia limbic encephalitis sensory neuronopathy most common in small cell lung cancer

Question 44

anti-LG1 symptoms

Answer 44

faciobrachial dystonic seizures hyponatremia memory probs myoclonus

Question 45

anti-Yo antibody symptoms, cancer

Answer 45

breast and pelvic cancer cerebellar degeneration --> ataxia

Question 46

most important prognostic biomarker for good prognosis in medulloblastoma?

Answer 46

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

Question 47

prognostic biomarker for poorest outcome in medulloblastoma?

Answer 47

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

Question 48

bubbly temporal mass in a kid with refractory seizures

Answer 48

DNET - glioneural elements on path resect it!

Question 49

IDH positive + 1p/19q codeletion

Answer 49

oligodendroglioma

Question 50

p53 + IDH mutation

Answer 50

astrocytomas

Question 51

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

Answer 51

DIPG. radiation only

Question 52

intracellular reticulin deposition

Answer 52

PXA