Pheochromocytoma due to unilateral adrenal medullary hyperplasia

Clinical Endocrinology
G QuptyR Luboshitzky

Abstract

We describe two male patients, aged 17 and 47 years, with clinical and biochemical features of pheochromocytoma. Both patients had normal-sized adrenal glands on abdominal CT scan and abnormal unilateral uptake of I-123 metaiodobenzylguanidine (MIBG) on scintigraphy. The surgical adrenalectomy revealed normal macroscopic glands in both patients. Histological examination showed adrenal medullary hyperplasia with adrenal cortico-medullary ratios of 2:1 and 4:1. Unilateral adrenalectomy resulted in amelioration of symptoms and normalization of catecholamines excretion. DNA examination for RET protooncogene revealed no mutations in exons 10, 11, 13, 14 and 16. Our results suggest that diffuse adrenal medullary hyperplasia may be the initial pathological change in the adrenal gland leading, subsequently, to the development of nodular hyperplasia and adrenal medullary tumor. These results indicate that the syndrome of pheochromocytoma may occur as an unilateral adrenal medullary hyperplasia in patients without evidence for multiple endocrine neoplasia.

Citations

Jan 12, 2002·Clinical Nuclear Medicine·Hendrikus H BoersmaMarinus J P G Van Kroonenburgh
Nov 25, 2003·AJR. American Journal of Roentgenology·Michael A BlakePeter F Hahn
Sep 11, 2013·JSLS : Journal of the Society of Laparoendoscopic Surgeons·Miguel Ruiz MarínAntonio Albarracín Marín Blázquez
Dec 10, 2014·World Journal of Surgical Oncology·Anna BabinskaKrzysztof Sworczak
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Mar 12, 2005·Arquivos brasileiros de endocrinologia e metabologia·Maria Adelaide A PereiraAntonio Marmo Lucon

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