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Reversible sellar enlargement due to growth hormone‐releasing hormone production by pancreatic endocrine tumors in an acromegalic patient with multiple endocrine neoplasia type I syndrome
Author(s) -
Ramsay Jennifer A.,
Kovacs Kalman,
Asa Sylvia L.,
Pike Malcolm J.,
Thorner Michael O.
Publication year - 1988
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19880715)62:2<445::aid-cncr2820620233>3.0.co;2-5
Subject(s) - acromegaly , medicine , endocrinology , somatotropic cell , somatostatin , endocrine system , growth hormone–releasing hormone , pituitary adenoma , pancreatic polypeptide , pancreas , multiple endocrine neoplasia , pituitary tumors , pancreatic tumor , hormone , adenoma , pituitary gland , glucagon , cancer , growth hormone , pancreatic cancer , biology , biochemistry , gene
A 28‐year‐old woman presented with hypoglycemia and acromegaly associated with pituitary sellar enlargement. Preoperative plasma levels of insulin and growth hormone (GH) were markedly elevated and there was mild hyperprolactinemia. Laboratory tests suggested hyperparathyroidism. Partial pancreatectomy was performed and two tumors were found. Morphologic examination revealed two well‐differentiated pancreatic endocrine neoplasms with distinct histologic, immunohistochemical, and ultra‐structural features. Immunoreactivity for insulin was present in the larger tumor; the smaller tumor contained glucagon, gastrin, somatostatin, and pancreatic polypeptide. Both neoplasms demonstrated growth hormone‐releasing hormone (GRH) immunopositivity and released GRH in vitro. Subsequent studies confirmed abnormally elevated preoperative plasma levels of GRH. Postoperatively, blood glucose, insulin, GRH, and GH normalized and there was regression of acromegalic features with significant reduction in sellar size. The clinicopathologic findings indicate that, in patients with multiple endocrine neoplasia type I (MEN‐I), GRH production by pancreatic tumors can stimulate hypophysial somatotrophs resulting in GH excess and acromegaly due to a reversible pituitary lesion, most likely somatotroph hyperplasia.