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neurocutaneous 03

Topic: Pathology

Created on Friday, February 13 2009 by gliageek

Last modified on Friday, February 13 2009.


Courtesy of Dr. Mark Cohen
This tumor is most closely associated with which of the following syndromes?

 
        A) Cowden diseaae
 
        B) Tuberous sclerosis
 
        C) Neurofibromatosis, type 2
 
        D) Von Hippel-Lindau
 
        E) Neurofibromatosis, type 1
 

 


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This question was created on February 13, 2009 by gliageek.
This question was last modified on February 13, 2009.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Cowden diseaae

This answer is incorrect.


Dysplastic gangliocytoma of the cerebellum (L'hermitte-Duclos disease) represents the CNS manifestation of Cowden disease  (See References)

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B) Tuberous sclerosis

This answer is correct.


Subependymal giant cell tumors are one of the cardinal manifestations of tuberous sclerosis.  (See References)

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C) Neurofibromatosis, type 2

This answer is incorrect.


Schwannomas, menigiomas, and ependymomas characterize NF2  (See References)

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D) von Hippel-Lindau

This answer is incorrect.


Hemangioblastomas are most closely associated with vHL.  (See References)

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E) Neurofibromatosis, type 1

This answer is incorrect.


Within the CNS, pilocytic astrocytomas of the optic nerve and chiasm are most closely associated with NF1  (See References)

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References:

1. Graham, D.I., and Lantos, P.L. (2002). Greenfield's Neuropathology, 7th ed. Arnold Press, New York. (ISBN:0340742313)Advertising:
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pathology
neurocutaneous 03
Question ID: 021309212
Question written by gliageek. (C) FrontalCortex.com 2006-2009, all rights reserved. Created: 02/13/2009
Modified: 02/13/2009
Estimated Permutations: 120

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