A rare association between acromegaly and pheochromocytoma

Department of Surgery, Division of General Surgery, University of Alberta, Cross Cancer Institute, Edmonton, Alberta, Canada.
The American Journal of Surgery (Impact Factor: 2.29). 09/2001; 182(2):185-7. DOI: 10.1016/S0002-9610(01)00678-X
Source: PubMed


The occurrence of multiple endocrine tumors is rare; however, they may be found with hereditary diseases such as multiple endocrine neoplasia (MEN). The endocrine tumors involved with these diseases are well documented. We present a case of a patient with a pheochromocytoma and a growth hormone (GH) secreting pituitary adenoma. This association is not described with any of the known MEN syndromes. The association may be a cross-over MEN syndrome or a secondarily induced GH-secreting pituitary adenoma from a pheochromocytoma producing growth hormone releasing hormone (GHRH) instead of catecholamines.

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    ABSTRACT: A 59-year old man, who had a hypertensive crisis during transsphenoidal surgery for a pituitary macroadenoma, was subsequently found to have an adrenal pheochromocytoma. A total of twenty-five cases describing the coexistence of a pituitary adenoma and pheochromocytoma have been reported in the literature over the past 40 years. Among pituitary tumors, acromegaly has been the most common. In an effort to identify the relationship between the two tumors, multiple theories have been suggested including fortuitous association, overlap or variants of MEN syndromes, and ectopic production of a trophic hormone by the pheochromocytoma. The high risk of mortality and morbidity associated with undiagnosed pheochromocytoma warrants careful attention to the possibility of such a coexistence.
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    ABSTRACT: Ectopic growth hormone-releasing hormone (GHRH)-secreting tumors are rare and cause acromegaly with somatotroph hyperplasia. We report a case of acromegaly secondary to GHRH secretion by an incidentally discovered pheochromocytoma in a normotensive patient. A 23-year-old man presented with signs and symptoms of acromegaly. Laboratory evaluation confirmed the diagnosis and magnetic resonance imaging (MRI) revealed a sellar mass which was thought to be a macroadenoma and surgically resected. The patient was not cured and medical treatment was indicated. An abdominal ultrasound performed before initiation of medical treatment showed a solid/cystic lesion superiorly to the right kidney. An abdominal MRI confirmed an adrenal tumor. Hormonal workup of the adrenal incidentaloma revealed elevated urinary catecholamine and total metanephrines findings strongly suggestive of a pheochromocytoma. Acromegaly was then suspected to be due to ectopic secretion of GHRH by the tumor. Patient underwent surgical resection and histopathologic examination confirmed a pheochromocytoma which stained positively for GHRH. Also, review of the pituitary specimen confirmed somatotrophic hyperplasia. Genetic analysis of the ret proto-oncogene showed no mutation. Pituitary MRI was repeated 10 months after pheochromocytoma resection and revealed a slightly enlarged pituitary and partial empty sella. The diagnosis of acromegaly caused by ectopic production of GHRH is a challenging task. A careful histopathological examination of the surgically excised pituitary tissue has a key role to arouse the suspicion and guide the investigation of a secondary cause of acromegaly.
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