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Pediatric Brain Tumor Pathology 01

Topic: Pathology

Created on Sunday, February 24 2008 by jdmiles

Last modified on Sunday, February 24 2008.

A developmentally delayed 4 year-old male presents with several months of worsening nausea and vomiting and seizures. A head CT reveals a large mass with areas of calcification. The mass is removed surgically. The image above shows an H&E stain of a section taken from the mass. Which of the following statements about this mass is true?

 
        A) This patient likely has neurofibromatosis type I
 
        B) This patient likely has neurofibromatosis type II
 
        C) This tumor is classified as WHO Grade III
 
        D) This patient likely has tuberous sclerosis
 
        E) This is an oligodendroglioma
 

 


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This question was created on February 24, 2008 by jdmiles.
This question was last modified on February 24, 2008.

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

ANSWERS AND EXPLANATIONS




A) This patient likely has neurofibromatosis type I

This answer is incorrect.


This tumor is associated with tuberous sclerosis, not neurofibromatosis.  (See References)

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B) This patient likely has neurofibromatosis type II

This answer is incorrect.


This tumor is associated with tuberous sclerosis, not neurofibromatosis.  (See References)

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C) This tumor is classified as WHO Grade III

This answer is incorrect.


This is a slow-growing, WHO Grade I tumor.  (See References)

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D) This patient likely has tuberous sclerosis

This answer is correct.


This tumor is a subependymal giant cell astrocytoma (SEGA). These tumors are typically long and sausagelike in appearance, often occurring in the lateral ventricle. They may cause hydrocephalus by obstructing the foramen of Munro. They are the most frequent neoplasm in children with tuberous sclerosis complex (TSC), and most SEGAs are associated with TSC. Calcification is typical. This is a slow-growing, WHO Grade I tumor. Pathologic appearance is as shown, with many astrocytes with abundant cytoplasm, often arranged in pseudorosettes. Some tumor cells may appear neuronal, and have prominent nucleoli.  (See References)

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E) This is an oligodendroglioma

This answer is incorrect.


This tumor does not have an appearance typical of an oligodendroglioma. Oligodendrogliomas classically have a "fried egg" appearance of dense cells.  (See References)

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References:

1. DeBiasi, R.L., Solbrig, M.V., and Tyler, K.L. (2004). Viral infections. In Bradley, W.G., Daroff, R.B., Fenichel, G.M., and Jankovic, J. (Eds.). Neurology in Clinical Practice, Fourth Edition. Butterworth Heinemann, Philadelphia, pp. 1515-1543 (ISBN:0750674695).Advertising:
2. Lopes, M.B., Altermatt, H.J., Scheithauer, B.W., Shepherd, C.W., and VandenBerg, S.R. (1996). "Immunohistochemical characterization of subependymal giant cell astrocytomas." Acta Neuropathol, 91(4) 368-75. (PMID:8928613)
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pathology
Pediatric Brain Tumor Pathology 01
Question ID: 022408120
Question written by J. Douglas Miles, (C) 2006-2009, all rights reserved.
Created: 02/24/2008
Modified: 02/24/2008
Estimated Permutations: 60480

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