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Neurocutaneous Syndromes 01

Topic: Adult

Created on Saturday, February 17 2007 by jdmiles

Last modified on Saturday, February 17 2007.

Which of the following is characteristically associated with neurofibromatosis type 1?

 
        A) Facial angiofibromas
 
        B) Shagreen patches
 
        C) Retinal astrocytoma
 
        D) Paucity or absence of cutaneous lesions
 
        E) Malignant transformation of tumors
 

 


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This question was created on February 17, 2007 by jdmiles.
This question was last modified on February 17, 2007.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) facial angiofibromas

This answer is incorrect.


Facial angiofibromas are not characteristic of NF1. They are associated with tuberous sclerosis.   (See References)

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B) shagreen patches

This answer is incorrect.


Shagreen patches are not characteristic of NF1. They are associated with tuberous sclerosis.   (See References)

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C) retinal astrocytoma

This answer is incorrect.


Retinal astrocytomas are not characteristic of NF1. They are associated with tuberous sclerosis.   (See References)

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D) paucity or absence of cutaneous lesions

This answer is incorrect.


Cutaneous lesions are characteristic of NF1. Lesions include cafe au lait spots, molluscum fobrosum (cutaneous tumors), and freckling in the axillae and groin. A paucity or absence of cutaneous lesions is typical of neurofibromatosis type 2 (NF2).   (See References)

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E) malignant transformation of tumors

This answer is correct.


Patients with NF1 and plexiform neurofibromas have a lifetime risk of about 5% for malignant transformaiton.   (See References)

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References:

1. Neurofibromatosis Fact Sheet, NINDS. NIH Publication No. 06-2126
2. Victor, M., and Ropper, A.H. (2001). Adams and Victor's Principles of Neurology, 7th Edition. McGraw-Hill, New York. Pp. 1069-1076.
3. Santos, C.C., Miller, V.S., and Roach, E.S. (2004). Neurocutaneous syndromes. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, 4th Edition. Butterworth Heinemann, Philadelphia. Pp. 1867-1900.
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adult
Neurocutaneous Syndromes 01
Question ID: 021707087
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/17/2007
Modified: 02/17/2007
Estimated Permutations: 84000
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