Medullary carcinoma of the thyroid, pancreatic nesidioblastosis and microadenosis, and pancreatic polypeptide hypersecretion: a new association and clinical and hormonal responses to long-acting somatostatin analog SMS 201-995.

Abstract	We describe a 63-yr-old man with disseminated medullary carcinoma of the thyroid and pancreatic nesidioblastosis and microadenosis with pancreatic polypeptide (PP) hypersecretion. His major symptoms were watery diarrhea, flushing, and abdominal bloating; these and the elevated plasma PP levels did not change after resection of the distal two thirds of the pancreas, which contained a 2-cm mass of nesidioblastotic tissue. Postoperatively, a long-acting somatostatin analog, SMS 201-995 (100 micrograms/day), normalized PP secretion acutely and chronically (7 months) and ameliorated his symptoms. The analog had no side-effects and did not alter glucose tolerance, calcitonin hypersecretion, or growth of the medullary carcinoma, but it did inhibit GH secretion. After withdrawal from therapy for 1 month, PP hypersecretion and all symptoms except diarrhea recurred. The coexistence of medullary carcinoma of the thyroid and PP cell nesidioblastosis represents a new variant of the overlap syndromes between multiple endocrine neoplasia types I and II. Patients with medullary carcinoma and unexplained watery diarrhea should have fasting gastroenteropancreatic hormone assays done to screen for a potential gastrointestinal or pancreatic origin for the diarrhea.
Authors	T W Jerkins, H S Sacks, T M O'Dorisio, S Tuttle, S S Solomon
Journal	The Journal of clinical endocrinology and metabolism (J Clin Endocrinol Metab) Vol. 64 Issue 6 Pg. 1313-9 (Jun 1987) ISSN: 0021-972X [Print] United States
PMID	2883196 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, Non-P.H.S., Research Support, U.S. Gov't, P.H.S.)
Chemical References	Peptides Somatostatin Calcitonin Octreotide
Topics	Calcitonin (metabolism) Carcinoma (complications, pathology) Eating Humans Immunochemistry Liver Circulation Male Middle Aged Octreotide Pancreas (abnormalities, metabolism, pathology) Pancreatic Diseases (complications, pathology) Peptides (blood, metabolism) Portal Vein Somatostatin (analogs & derivatives, therapeutic use) Thyroid Neoplasms (complications, pathology)

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