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Neurocutaneous 04

Topic: Pathology

Created on Monday, February 16 2009 by gliageek

Last modified on Monday, February 16 2009.

Courtesy of Dr. Mark Cohen

While removing this tumor from the spinal cord of a 8 year old boy, the surgeon reported encountering several gritty meningeal lesions, each less than a mm in diameter. The most likely diagnosis for this child is?

A) Schwannomatosis

B) Neurofibromatosis, type 2

C) Von Hippel-Lindau disease

D) Carney complex

E) Tuberous sclerosis

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This question was created on February 16, 2009 by gliageek.
This question was last modified on February 16, 2009.

ANSWERS AND EXPLANATIONS

A) Schwannomatosis

This answer is incorrect.

This possibly "form fruste" of NF2 does not include ependymomas or meningiomas (See References)


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B) Neurofibromatosis, type 2

This answer is correct.

When NF2 presents in childhood, it is commonly as intramedullary ependymoma. The gritty lesions were psammomatous meningiomas (See References)


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C) von Hippel-Lindau disease

This answer is incorrect.

Intramedullary tumors are hemangioblastomas, and are very rare in childhood (See References)


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D) Carney complex

This answer is incorrect.

Psammomatous melanotic schwannoma may be a manifestation of Carney complex, but ependymoma is not (See References)


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E) Tuberous sclerosis

This answer is incorrect.

TS does not involve meninges or predispose to ependymal tumors (See References)


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References:

1. Love, S., Louis, D.N., and Ellison, D.W. (2008). Greenfield's Neuropathology, 8th Edition. Oxford University Press, USA (ISBN:0340906820)

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pathology
Neurocutaneous 04
Question ID: 021609100
Question written by gliageek. (C) FrontalCortex.com 2006-2009, all rights reserved. Created: 02/16/2009
Modified: 02/16/2009
Estimated Permutations: 120