Neurooncology Flashcards
1
Q
Opsoclonus-myoclonus
A
- paraneoplastic, related to breast, ovarian, SC lung, and neuroblastoma (kids)
- Adults may have anti-Ri antibodies
Symptoms:
1. rapid irregular high-amplitude conjugate eye movements in any direction
2. ataxia
3. myoclonic jerks
Treatment:
- ACTH (kids)
2
Q
Brain tumors rarity, survival
A
Adults
1. Glioma (most common primary)
- astrocytoma (Grade II) - 5-10 years
- Anaplastic astrocytoma (Grade III) -2-3 years
- GBM (Grade IV, 50% of gliomas) - <1 yr
Children
Pilocytic astrocytoma (Grade 1) - most common glioma in kids
3. Ependymoma
3
Q
- Perinecrotic pseudo-pallisading
- Fried egg
- perivascular pseudorosettes
- Homer-wright rosettes
- Rosenthal fibers
- Psammoma bodies
A
- GBM
- Oligodendrogliomas
- ependymomas
- Medulloblastoma
- Alexander’s disease, pilocytic astrocytoma, pleomorphic xanthoastrocytoma, chronic reactive gliosis
- Meningioma
4
Q
Temozolumide
A
- Alkylating agent chemotherapy used as adjuvant treatment for brain tumors in addition to resection and radiation
- MGMT-methylated tumors have less MGMT which repairs DNA, making alkylating chemo more effective
5
Q
Bevacizumab
A
- monoclonal AB that binds VEGF, anti-angiogenic.
- may decrease edema and reduce the need for steroids
6
Q
- MGMT-methylation
- IDH1
- p53 mutation
- 1p19q deletion
Meaning? Prognosis?
A
- MGMT-methylated tumors have less MGMT which repairs DNA, making alkylating chemo more effective. Positive methylation = better prognosis
- IDH1 codes for isocitrate dehydrogenase. Mutations usually seen in lower-grade gliomas, and presence in a higher-grade glioma suggests it progressed from a lower stage. This mutation is associated with an improved prognosis
- p53 mutation in 5-% of Grade III gliomas
- found in most oligodendrogliomas, associated with better response to treatment and prognosis
7
Q
Paraneoplastic syndromes
- Anti-Hu (ANNA1), Anti-Yo, Anti-Tr
- Anti-CRMP-5
- Anti-recoverin, Anti-CAR
- Anti-Hu (ANNA1)
- Anti-Hu, Anti-CRMP-5
A
- Paraneoplastic cerebellar degeneration, (Breast/ovarian, SC lung, lymphomas respectively) - cerebellar signs
- Paraneoplastic optic neuropathy (lung) - vision loss
- Paraneoplastic retinal degeneration (SC lung, thymoma, RC carcinoma, melanoma) - scotomas, vision loss, disc swelling
- Paraneoplastic subacute sensory neuropathy (SC lung)
- Paraneoplastic chorea (lung) - choreoathetosis
8
Q
Pleomorphic xanthoastrocytoma
Appearance, presentation, treatment?
A
- WHO grade II, well-demarcated superficial cortex tumor, usually temporal lobes, usually children
- cyst w/ mural nodule (similar to pilocytic astrocytoma)
- often presents as focal epilepsy
- often resectable
9
Q
Ganglioglioma
Cells? Presentation? Treatment?
A
- mix of glia and neurons
- Usually temporal lobe in kids, presents as seizures. Usually well circumscribed with mural nodule
- Glial component has glial fibrillary acid protein (GFAP) positivity
- Grade I, resectable, good prognosis
10
Q
Neurocytoma
Cells? Presentation? Treatment?
A
- neural cells reactive w/ synatrophysin, 30s-50s, intraventricular
- looks like oligodendrocytoma but does not react with glial markers
- Grade II w/ good prognosis, resection
11
Q
Meningioma
Cells? Presentation? Treatment?
A
- monomorphic oval nuclei cells, psommoma bodies - 50% w/ loss of chromosome 22
- elderly, meningeal (dural), compression, seizures
- resection +/- radiation
12
Q
Hemangioblastoma
- Description? Presentation? Treatment?
A
- benign, highly vascular tumor, lots of capillaries w/ vaculated cytoplasm
- most common primary cerebellar neoplasm in adults, can have mass effect and CSF obstruction
- can secrete EPo leading to secondary polycythemia
- WHO grade I, surgical resection
- ASSOCIATED WITH von Hippel-Lindau disease related to VHL gene mutation (25% of cases)
13
Q
Medulloblastoma
- Description? Presentation? Treatment?
A
- hypercellular small blue cells, homer-wright pseudorosettes
- rapidly growing invasive tumor seen mostly in children
- posterior fossa symptoms. May metastasize to spine
- resection, radiation, chemo
- prognosis: Worse w/ subtotal resection, brainstem invasion, <3 years old, large cell variant, N-myc transcription factor amplification
14
Q
Colloid cyst
A
benign cyst in ventricle near foramen of monroe - may cause obstruction w/ headaches, drop attacks
single membrane layer with thick fluid. Resectable
15
Q
Epidermoid/dermoid cysts
A
- ectopic ectodermal tissue
- epidermoid: young adults, cerebellopontine angle, fluid filled but with RESTRICTED DIFFUSION on DWI
- dermoid: children, cerebellar vermis, parasellar, parapontine, or lumbosacral spinal canal. Pearly white structure, contain hair follicles and sweat glands. Can rupture and cause a chemical meningitis
