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Flashcards in brain neoplasm and peds neuro Deck (13)
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1

What is the most common malignant brain tumor in children?

medulloblastoma; can send "drop mets" to the spinal cord. due to a primitive neuroectodermal tumor

2

What cells give rise to meningioma?

arachnoid cells. may have psammoma bodies

3

What are the most common sources for brain mets?

lung, renal cell carcinoma, melanoma, breast, colorectal

4

What are the diagnostic criteria for neurofibromatosis type 1?

chromosome 17. pts have at least 2 of the following:
1. >5 cafe au lait spots 1 neurofibromas
3. axillary or inguinal freckling
4. optic glioma
5. >1 iris hamartomas (lisch nodules)
6. bone lesions
7. first degree relative with NF1
COFFINS: cafe au lait spots, optic glioma, freckles, family hx, iris hamartomas, neurofibromas, skeletal lesions

5

What are the functional ramifications of NF1?

may hae severe functional limits in gait and movement due to nonunion of bone fragments (pseudoarthrosis) and fractures during development
may have vision loss from optic glioma

6

What are potential complications of NF1?

incr. risk of pheochromocytoma, seizures, peripheral neuropathy, and primary CNS tumors

7

Sturge-weber syndrome:

STURGE: sporatic (non-inherited), port-wine stain, unilateral, retardation, incr risk of glaucoma, GNAQ gene, epilepsy due to ipsilateral leptomeningeal angioma

8

tuberous sclerosis

HAMARTOMAS: hamartomas in CNS and skin, angiofibromas, mitral regurgitation, ash-leaf spots, cardiac rhabdomyoma, (tuberous sclerosis, autosomal dOminant), MR, renal angiomyolipoma, seizures, shagreen patches, incr. incidence of subependymal astrocytomas

9

von Hippel Lindau

hemangiomas in skin, mucosa, organs, bilat renal cell carcinoma, hemangioblastoma in retina, brain stem, and cerebellum, pheochromocytoma.

10

When should LP be performed after febrile seizure?

any child

11

What enzyme is deficient in Tay-Sachs? How do you distinguish from Nieman Pick?

hexosaminidase A; GM2 ganglioside accumulates. Both Tay-sachs and Nieman pick have cherry red macula but Tay-Sachs does NOT have hepatosplenomegaly

12

What is a major complication of lower neural tube defects and how is it treated?

hydrocephalus; often requires shunting

13

What other tumors are children with retinoblastoma tumor suppressor mutations at risk for?

osteosarcoma