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Pediatrics General 957f6614

A 3 year old child is referred to a major medical center because of an abdominal mass arising from his right adrenal gland. Biopsy of the lesion demonstrates sheets of small cells with hyperchromatic nuclei containing occasional pseudorosettes composed of circles of tumor cells with central young nerve fibers arising from the tumor cells. Which of the following oncogenes is associated with this patient's tumor?

A
erb-B2
B
c-myc
C
L-myc
D
N-myc
High-Yield Explanation
The tumor is a neuroblastoma, which is one of the principal forms of cancer in children. Neuroblastoma typically occurs before age 5, with many presenting before age 2. Neuroblastoma can arise from neural crest cells throughout the body, but the adrenal medulla is the most common site. Homer-Wright pseudorosettes are circles of tumor cells with central young nerve fibers arising from the tumor cells. The oncogene associated with neuroblastoma is N-myc. erb-B2 is associated with breast, ovarian, and gastric carcinomas. c-myc is associated with Burkitt's lymphoma. L-myc is associated with small cell carcinoma of the lung. Ref: Graham D.K., Quinones R.R., Keating A.K., Maloney K., Foreman N.K., Giller R.H., Greffe B.S. (2012). Chapter 31. Neoplastic Disease. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.

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