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Acromegaly and Pheochromocytoma: A Multiple Endocrine Syndrome Caused by a Plurihormonal Adrenal Medullary Tumor^*

KEVIN A. ROTH, DARRELL M. WILSON, JAMES EBERWINE, RICHARD I. DORIN, KALMAN KOVACS, KLAUS G. BENSCH and ANDREW R. HOFFMAN

Nancy Pritzker Laboratory of Behavioral Neurochemistry, Stanford University School of Medicine Stanford, California 94305
The Departments of Psychiatry and Behavior Sciences, Stanford University School of Medicine Stanford, California 94305;
The Departments of Psychiatry, Stanford University School of Medicine Stanford, California 94305
The Departments of Medicine, Stanford University School of Medicine Stanford, California 94305
The Department of Pathology, Stanford University School of Medicine Stanford, California 94305
St. Michael’s Hospital, University of Toronto Toronto, Ontario M5B 1W8, Canada

Address all correspondence and requests for reprints to: Andrew R. Hoffman, M.D., Division of Endocrinology, Room S-005, Stanford University School of Medicine, Stanford, California 94305.

A 42-yr-old man with congestive heart failure and diabetes mellitus was found to have acromegaly and a pheochromocytoma. Serum GH-releasing hormone (GHRH) levels were elevated (2.34 ng/dl; normal, <0.02 ng/dl), suggesting that the acromegaly was caused by ectopic secretion of GHRH. Postmortem examination revealed that the right adrenal gland contained a pheochromocytoma in which GHRH was demonstrated by immunohistochemical studies. Gel permeation chromatography combined with the use of two GHRH antisera showed that GHRH-(l–44)-NH₂ was a predominant form of the hormone. When the RNA from the tumor was extracted and analyzed by Northern gel blotting, two mRNA species were identified, with transcripts corresponding to 1600 and 780 base pairs. The pituitary gland was enlarged, but no distinct adenoma was found. Diffuse and nodular hyperplasia of somatotrophs in some areas resembling adenoma was identified on histological examination. These findings indicate that GH excess accompanied by somatotroph hyperplasia and acromegaly were secondary to a pheochromocytoma which secreted not only catecholamines but also GHRH.

^* This work was supported in part by grants from the NIH (AG-01312 and AM-36054), the NIMH (MH-23861), and the American Institute for Cancer Research.

Daland Fellow of the American Philosophical Society and recipient of National Research Service Award AM-07407.

Received May 2, 1986.

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