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Neuro-oncology > neurocutaneous disorders” (or phakomatoses) > Flashcards

neurocutaneous disorders” (or phakomatoses) Flashcards

1
Q

NF1

A

Optic Glioma
Astrocytomas
Neurofibroma

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2
Q

NF2

A

Bilateral vestibular Shawnnoma
Meningioma
Epndymoma
Astrocytomas

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3
Q

schwannomatosis

A

multiple schwannomas, infrequently meningiomas

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4
Q

tuberous sclerosis

A

subependymal giant cell astrocytoma (SEGA)

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5
Q

von Hippel-Lindau syndrome

A

hemangioblastoma

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6
Q

Li-Fraumeni syndrome

A

children: medulloblastoma
adult: astrocytoma, PNET

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7
Q

Cowden syndrome

A

dysplastic cerbellar gangliocytoma

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8
Q

constitutional mismatch repair deficiency syndrome

A

ultrahypermutated malignant gliomas, CNS embryonal tumors

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9
Q

familial adenomatous polyposis 1 (p.651) (FAP1) + brain tumors (BTP2)

A

medulloblastoma WNT-activated

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10
Q

nevoid basal cell carcinoma syndrome (Gorlin syndrome

A

desmoplastic/nodular subtype of medulloblastoma

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11
Q

rhabdoid tumor predisposition syndrome (RPTS)

A

atypical teratoid/rhabdoid tumors (AT/RTs)

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12
Q

Carney complex

A

malignant melanotic nerve sheath tumor, schwannoma, PitNET/adenoma

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13
Q

DICER1 syndrome

A

metastatic pleuropulmonary blastoma; pineoblastoma; embryonal tumor with multilayered rosettes; pituitary blastoma; primary intracranial sarcoma, DICER1-mutant

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14
Q

familial paraganglioma syndromes

A

paraganglioma (including pheochromocytoma)

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15
Q

melanoma-astrocytoma syndrome

A

astrocytoma, nerve sheath tumors

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16
Q

familial retinoblastoma

A

pineoblastoma

17
Q

BAP1 tumor predisposition syndrome

A

meningioma

18
Q

Fanconi anemia

A

medulloblastoma

19
Q

ELP1-medulloblastoma syndrome

A

medulloblastoma SHH-activated

20
Q

NF2 MCC intramedullary spinal tumor

A

Epndymoma

21
Q

NF1 MCC intrameduallry spinal cord tumor

A

Astrocytomas

22
Q

Schwannoma vs neurofibroma

A

Displace axon centrifugal > shwannoma

centripetal engulf nerve > neurofibroma

23
Q

Schwannoma vs. neurofibroma. Which one has malignancy potential

A

Neurofibroma

Neuro-oncology (13 decks)

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