Neuro oncology Flashcards
choroid plexus papilloma
enhancing in ventricle if kids, in 3rd or 4th ventricle in adults
-looks like normal choroid plexus on path
papilloma = grade 1
carcinoma. grade iii - Li-Fraumeni syndrome
-hydrocephalus Sx
colloid cyst
hyperdense on CT (protinaeous fluid)
-roof of 3rd ventricle
-ASx usually or transient LOC (obstructive hydrocephalus)
subependymal giant cell astrocytoma
tuberous sclerosis
central neurocytoma
off of broad attachment to septum pellucidum
SC tumors- extramedullary, intradural and extradural
intradural/outside SC: meningioma, nerve sheath tumors
Extradural: metastases
SC tumors -intramedullary
ependymoma - ovale/sausage shaped mass
astrocytoma - pilocytic astrocytoma, anaplastic astrocytoma, glioblastoma, H327K midline glioma
lymphoma
hemangioblastoma [(multiple (VHL-cerebellum)]
-masses blood vessels on path - cyst with mural nodule
Pineal tumors
1st -serum tumor markers - AFP, bhCP
-usually needs biopsy
Germ cell - germinoma - engulfed calcifications
-no surgery unless mature teratoma, use radiation
pineal cell tumors - peripheral calcifications (blast calcifications out of the way)
-pineocytoma/pineoblastoma (blastoma = grade IV tumors in kids)
grade 1 glioma
-pilocytic astrocytoma most common
-can enhance or having enhancing mural nodule, cyst; well circumscribed
-cerebellum
diffuse gliomas
- Astrocytoma
-IDH mutant
-p53, ATRX, p16 - Oligodendroglioma -diffuse infiltrating
-IDH mutation
-IDH/1p19q co-deletion best prognosis
-40s-50s, 3:2 males
-frontal lobe predominant
Grade II - not enhancing; grade III-enhancing
-mass effect, variable enhancement
- GBM, IDH1 WT
histo: -perinecrotic pseudopalisading, necrosis, endothelial hyperplasia
-heterogenous + necrotic
Tx: surgical resection then RT + temozolamide
-MGMT methylated tumors-respond better to temozolamide b/c MGMT is not antagonizing to temozolamide
-recurrent tumor - Tx-Bevacizumab - VGEF
diffuse midline glioma
grade iv
H3K27M mutant
CNS lymphoma
can be multifocal
homogenously enhancing
high dose methodexate induction then consolidation /
paraneoplastic disorders
Anti-Ri-ANNA 2 - opsoclonus myoclonus
Movement disorders:
Anti Ma
Anti DPP6
ANNA1 (Hu), CASPR2,
collapsin response mediator protein-5 (CRMP-5, also known as CV2),
LGI1 syndromes
GFAP
opsoclonus myoclonus - neuroblastoma in kids; breast, ovarian, SCLC
-Anti-Ri - breast cancer, SCLC
cerebellar degeneration - anti-Yo (anti-Purkinje cell ab) - ovarian, breast, Hodkin’s lymphoma, SCLC
-weeks-months ataxia, nystagmus, dysarthria, hearing loss
-path: loss Purkinje cells, atrophy cerebellum
anti-CRMP-5 - optic neuropathy; chorea - lung cancer
retinal degeneration - anti-recoverin/anti-CAR - SCLC, thymoma, RCC, melanoma
- disc swellings, scotomas, nocturnal vision loss
Anti-Hu-ANNA1 - subacute sensory neuropathy/neuronopathy; chorea - -SCLC
LEMS-P/Q VGCC- SCLC
Anti-Tr-lymphomas-young males
neuroblastoma
opsoclonus myoclonus
-myoclonic jerks+ataxia
Tx: ACTH
circumscribed gliomas
- Pilocytic astrocytoma - grade I, in cerebellum, contrast enhancing mural nodule, cystic
-optic nerve - NF1
Tx: surgery cures - Pleomorphic Xanthoastrocytoma - grade II/III- temporal lobes, superficial, focal epilepsy
-enhancing mural nodule, cyst
-histo: bizarre nuclear pleomorphism, lipidized cells, eosinophilic granular bodies, intercellular reticulin deposit
-can undergo malignancy transformation 20% - Subependymal Giant Cell Astrocytoma - grade I - 3rd or lateral ventricles (ependymoma - 4th ventricle)
-TSC only
Histo: -calcifications
-candle gutterings
High Grade Astrocytoma with Piloid Features
Chordoid Glioma
Atroblastoma
Glioneuronal, neuronal tumors
Gangliocytoma
Ganglioglioma
DNET