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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) Von Hippel-Lindau disease
 
        B) Schwannomatosis
 
        C) Neurofibromatosis, type 2
 
        D) Tuberous sclerosis
 
        E) Carney complex
 

 


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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) von Hippel-Lindau disease

This answer is incorrect.


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

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B) Schwannomatosis

This answer is incorrect.


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

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C) 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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D) Tuberous sclerosis

This answer is incorrect.


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

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E) 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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References:

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

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