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Neurocutaneous 01

Topic: Pathology

Created on Saturday, February 7 2009 by gliageek

Last modified on Saturday, February 7 2009.


Courtesy of Dr. Mark Cohen

Which of the following neurocutaneous syndromes is most closely associated with this neoplasm?

 
        A) Neurofibromatosis, type 2
 
        B) Tuberous Sclerosis
 
        C) Von Hippel-Lindau disease
 
        D) Sturge-Weber disease
 
        E) Neurofibromatosis, type 1
 

 


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This question was created on February 07, 2009 by gliageek.
This question was last modified on February 07, 2009.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Neurofibromatosis, type 2

This answer is incorrect.


NF2 is characterised by multiple schwannomas and meningiomas. Though NF2 may develop meningiomas involving the optic nerve, this is not particularly characteristic of the syndrome.  (See References)

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B) Tuberous Sclerosis

This answer is incorrect.


TS is characterised by multiple hamartomatous lesions both within and outside of the nervous system. The optic nerve is generally spared.  (See References)

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C) von Hippel-Lindau disease

This answer is incorrect.


VHL is characterised by multiple hemangioblastomas involving the nervous system. These have been reported to involve the optic nerve, but are quite rare.  (See References)

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D) Sturge-Weber disease

This answer is incorrect.


SWD is characterised by angiomatous vascular proliferation involving the meninges with accompanying cortical calcification. Involvement of the optic nerve is uncommon & would not lead to its expansion.  (See References)

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E) Neurofibromatosis, type 1

This answer is correct.


Although often referred to as "peripheral neurofibromatosis", children with neurofibromatosis type 1 often present with pilocytic astrocytomas of the optic nerve, which may be bilateral.  (See References)

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References:

1. Prayson, R.A., and Goldblum, J.R. (Eds.) (2005). Neuropathology. Elsevier Churchill Livingstone, Philadelphia. (ISBN:0443066582)Advertising:
2. Listernick, R., Ferner, R.E., Liu, G.T., and Gutmann, D.H. (2007). "Optic pathway gliomas in neurofibromatosis-1: controversies and recommendations." Ann Neurol, 61(3) 189-98. (PMID:17387725)
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pathology
Neurocutaneous 01
Question ID: 020709186
Question written by gliageek. (C) FrontalCortex.com 2006-2009, all rights reserved. Created: 02/07/2009
Modified: 02/07/2009
Estimated Permutations: 120

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