Acromegaly and pheochromocytoma: a multiple endocrine syndrome caused by a plurihormonal adrenal medullary tumor.
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, less than 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-(1-44)-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.
D000172:Acromegaly / Q000209:etiology*; D000310:Adrenal Gland Neoplasms / Q000378:metabolism* / Q000473:pathology; D000313:Adrenal Medulla / Q000378:metabolism*; D000328:Adult; D002395:Catecholamines / Q000378:metabolism; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism; D006801:Humans; D008297:Male; D009384:Paraneoplastic Endocrine Syndromes / Q000209:etiology*; D010673:Pheochromocytoma / Q000378:metabolism* / Q000473:pathology; D010903:Pituitary Gland, Anterior / Q000473:pathology
D002395:Catecholamines; D013007:Growth Hormone-Releasing Hormone