FrontalCortex, Inc. is a non-profit corporation dedicated to neurology education.

NeurOnc10

Topic: Imaging

Created on Sunday, March 3 2013 by gliageek

Last modified on Sunday, March 3 2013.


Courtesy of Dr. Mark Cohen
This tumor was removed from the posterior fossa of a 14 month old boy. Further investigation is indicated to look for which of the following syndromes?

 
        A) Neurofibromatosis 1
 
        B) Li-Fraumeni syndrome
 
        C) Tuberous sclerosis complex
 
        D) Neurofibromatosis 2
 
        E) Gorlin syndrome
 

 


Back to the question = Go back to the top of the page.
See another question like this one = Reload a different version of this question ().
Click here for a random question = Load a random question from the database.
Clone this question = Use this question as a template to create a totally NEW question.
Rate this question = Enter detailed rating for this question!
Average rating not yet available
= How users like you have rated this question.
This question was created on March 03, 2013 by gliageek.
This question was last modified on March 03, 2013.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) Neurofibromatosis 1

This answer is incorrect.


Optic pathway gliomas occur in about 15% of NF-1 patients, usually before age 7.  (See References)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this question
Average rating not yet available
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




B) Li-Fraumeni syndrome

This answer is incorrect.


Choroid plexus carcinoma is currently considered an LFS-defining tumor  (See References)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this question
Average rating not yet available
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




C) Tuberous sclerosis complex

This answer is incorrect.


Subependymal giant cell astrocytomas present in approximately 10% of patients with TSC  (See References)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this question
Average rating not yet available
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




D) Neurofibromatosis 2

This answer is incorrect.


Although rare, childhood spinal ependymomas may be the first indication of the presence of NF-2  (See References)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this question
Average rating not yet available
Please log in if you want to rate questions.

 

 

 

 

 

 

 

 

 




E) Gorlin syndrome

This answer is correct.


Desmoplastic medulloblastoma is commonly the first malignant manifestation of the nevoid basal cell carcinoma syndrome; some believe that its presence in a child less than 2 years old should be a major criterion for the diagnosis of NBCCS.  (See References)

Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this question
Average rating not yet available
Please log in if you want to rate questions.

 

 

 

 

References:

1. Amlashi, S.F., Riffaud, L., Brassier, G., and Morandi, X. (2003). "Nevoid basal cell carcinoma syndrome: relation with desmoplastic medulloblastoma in infancy. A population-based study and review of the literature." Cancer, 98(3) 618-24. (PMID:12879481)
2. Lo Muzio, L. (2008). "Nevoid basal cell carcinoma syndrome (Gorlin syndrome)." Orphanet J Rare Dis, 3 32. (PMID:19032739)
Back to the questionSee another question like this oneClick here for a random questionClone this question Rate this question
Average rating not yet available
Please log in if you want to rate questions.

 

FrontalCortex.com -- Neurology Review Questions -- Neurology Boards -- Board Review -- Residency Inservice Training Exam -- RITE Exam Review
imaging
NeurOnc10
Question ID: 030313163
Question written by gliageek. (C) FrontalCortex.com 2006-2009, all rights reserved. Created: 03/03/2013
Modified: 03/03/2013
Estimated Permutations: 120

User Comments About This Question:

0 user entries
Please log in if you'd like to add a comment.