Buzz Words Flashcards
1p19q deletion
Oligodendroglioma, better prognosis
IDH1 mutation
Low grade glioma
Treatment for glioblastoma
Surgical resection/debulking
Radiation
Temozolomide (alkylating agent)
P53 mutation
Anaplastic Astrocytoma
Mutation present in 50%
Grade iii , survival 2-3 years
Well demarcated tumor in temporal lobe (cortical) , causes focal epilepsy
PXA. Resectable, good prognosis but 15-20% become malignant
“Candle gutterings”
Masses around vents associated with SEGAs (TSC and young people)
Infiltrating tumor in frontal cortex of a middle aged person
Oligodendroglioma
Paraneoplastic syndrome: retinal degradation (antibody, tumor?)
Anti-recoverin (anti-CAR)
Small cell lung, renal cell, melanoma, thymoma
ganglioglioma
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
myxopapillary variant
a variant of ependymoma that arises from filum terminale, seen in adults, good prognosis (Grade I)
ependymoma genetics
assoc with 22q deletions and NF2
what 2 things are assoc with worse prognosis for ependymoma?
age of presentation younger than 3 years
drop mets
sheets of cells and perivascular pseudorosettes on path
ependymoma
ependymoma
3rd most common tumor in kids
infratentorial usually
perivascular pseudorosettes, sheets of cells
drop mets and <3 - worse prog
meningioma
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
psammoma bodies
meningioma
most common primary cerebellar tumor in adults
hemangioblastoma
hemangioblastoma presentation
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
hypercellular, small blue cells, Homer Wright rosettes
medulloblastoma - invasive cerebellar tumor. 20% of kid tumors
pineal gland tumors (pineocytoma vs pineoblastoma)
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
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.
colloid cyst
T1 signal, small enhancing rim
cyst that arises from ectopic ectodermal tissue, in young adults, from cerebellopontine angle, DWI diffusion restricting
epidermoid cyst
CSF intense on t2, restricted diffusion on DWI (distinguishes it from arachnoid cyst)
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
dermoid cyst
if ruptures –> chemical meningitis with granulomatous inflammation
where are arachnoid cysts typically seen
temporal lobes, meninges
where does Rathke’s cleft cyst occur
between anterior and posterior hypophysis
embryonal tumor that arises from pluripotential cells, seen in kids, more common in boys, posterior fossa
medulloblastoma
incr ICP, hydrocephalus, cerebellar findings
why should you get a spine MRI if you see medulloblastoma in cerebellum
can metastasize via CSF
poor prognosis in medulloblastoma
<3 years at onset
incomplete resection
brainstem involvement
mets
N-myc transcription factor amplification
refractory sz in kids - what tumor? grade?
DNET - cortical/juxtacortical, temporal lobe
WHO Grade I, resection curative
primary CNS lymphoma
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
tumor causing visual disturbances, kids and middle aged adults
craniopharyngioma
multicystic, benign, arise from Rathke’s pouch
mass effect –> optic chiasm, pituitary, hypothalamus, vents
lipid and cholesterol rich fluid inside
tx surgical resection
what is a chordoma and where do they occur
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
unilateral hearing loss, tinnitus, ataxia, circumscribed contrast enhancing tumor in cerebellopontine angle
vestibular schwannoma
brain mets - most common source?
- lung
- breast
- melanoma
80% supratentorial.
very rare to have mets from prostate, esophagus, other skin cancers
most common tumors of cerebellopontine angle
acoustic schwannoma
meningioma
epidermoid cyst (diffusion restricts, chemical meningitis)
man with MGUS and peripheral neuropathy. work-up?
anti-MAG antibody - (myelin associated glycoprotein)
paraneoplastic syndrome causing brainstem encephalitis, assoc with lung and breast cancer
Anti-Ri/ANNA-2
kid with cerebellar tumor, cystic with mural nodule. dx and prognosis?
pilocytic astrocytoma
grade I , slow growth + good prognosis
surgical resection, +/- radiation if can’t completely resect
assoc with NF1 (usually optic pathway)
oligodendroglioma vs central neurocytoma
both have fried eggs on histopath
oligos are +IDH mutation and +1p/9q codeletion
central neurocytomas - synaptophysin
most common cancer assoc with opsoclonus myoclonus syndrome in adults?c
small cell lung cancer
if anti-Ri/ANNA-2 –> breast cancer (second most common cancer)
pineal region tumor seen on MRI - next step?
check levels of AFP and b-HCG - if normal, proceed with biopsy.
resect mature teratomas only, not clear if this helps germ cell tumors
pineoblastoma management
get MRI spine - can seed neuroaxis
check CSF for malignant cells
resection, brain/spine radiation, chemo
anti-Hu (ANNA-1) symptoms
sensory predominant peripheral neuropathy
cerebellar ataxia
limbic encephalitis
sensory neuronopathy
most common in small cell lung cancer
anti-LG1 symptoms
faciobrachial dystonic seizures
hyponatremia
memory probs
myoclonus
anti-Yo antibody symptoms, cancer
breast and pelvic cancer
cerebellar degeneration –> ataxia
most important prognostic biomarker for good prognosis in medulloblastoma?
WNT activation –> 100% 5 year survival
(will not tank)
prognostic biomarker for poorest outcome in medulloblastoma?
MYC amplification
(May You Cherish….your time left)
bubbly temporal mass in a kid with refractory seizures
DNET -
glioneural elements on path
resect it!
IDH positive + 1p/19q codeletion
oligodendroglioma
p53 + IDH mutation
astrocytomas
girl with cranial nerve deficits and ataxia, hyperreflexia found to have large expansile brainstem mass. dx and tx?
DIPG. radiation only
intracellular reticulin deposition
PXA
