16 July 2014 - Case of the Week #319
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Case of the Week #319
Clinical History:
A 34 year old man with a history of multiple endocrine neoplasia type IIA presented with an adrenal gland mass.
Gross images:
Micro images:
What is your diagnosis?
Diagnosis:
Composite pheochromocytoma with ganglioneuroma
Discussion:
Immunostains were obtained:
Chromogranin
Neurofilament
S100
Chromogranin is positive in pheochromocytes, weak in ganglion cells and negative in Schwann cells. Neurofilament is positive in the ganglion and Schwann cells, and is negative in the pheochromocytes. S100 is positive in Schwann and sustentacular cells surrounding the pheochromocytes, and is negative in the ganglion cells.
Multiple endocrine neoplasia type IIA was first described by Sipple in 1961 (American Journal of Medicine 1961; 31:163). It is an autosomal dominant disorder due to mutation in the RET proto-oncogene, in which all patients have medullary thyroid carcinoma, 30-50% have pheochromocytoma and 10-15% have parathyroid hyperplasia. Differences between MEN IIA and IIB are that IIA patients lack mucosal neuromas and marfanoid features of IIB patients, IIA patients have a less virulent form of medullary thyroid carcinoma, and IIB patients only rarely have parathyroid hyperplasia (Medscape).
Composite pheochromocytoma of the adrenal medulla is rare, typically indistinguishable clinically and radiologically from pheochromocytoma (Endocr Pract 2014 Mar 18:1 [Epub ahead of print]). It is associated with catecholamine overproduction, and has a median tumor size of 7 cm (range, 2.5-13 cm). These composite tumors are only infrequently associated with MEN 2A syndrome (Am J Surg Pathol 1997;21:102, J Endocrinol Invest 2005;28:734).
Most composite tumors are nonaggressive, with recommended treatment similar to classic pheochromocytoma (Endocr Pract 2010;16:291).
Nat Pernick, M.D., President
and Shivani Thakore, Associate Medical Editor
PathologyOutlines.com, Inc.
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