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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) Tuberous sclerosis complex

B) Gorlin syndrome

C) Neurofibromatosis 2

D) Li-Fraumeni syndrome

E) Neurofibromatosis 1

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This question was created on March 03, 2013 by gliageek.
This question was last modified on March 03, 2013.

ANSWERS AND EXPLANATIONS

A) Tuberous sclerosis complex

This answer is incorrect.

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

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B) 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)

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C) Neurofibromatosis 2

This answer is incorrect.

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

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D) Li-Fraumeni syndrome

This answer is incorrect.

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

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E) Neurofibromatosis 1

This answer is incorrect.

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

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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)

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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