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.
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.
D002116:Calcitonin / Q000378:metabolism; D002277:Carcinoma / Q000150:complications* / Q000473:pathology; D004435:Eating; D006801:Humans; D007120:Immunochemistry; D008102:Liver Circulation; D008297:Male; D008875:Middle Aged; D015282:Octreotide; D010179:Pancreas / Q000002:abnormalities* / Q000378:metabolism / Q000473:pathology; D010182:Pancreatic Diseases / Q000150:complications* / Q000473:pathology; D010455:Peptides / Q000097:blood / Q000378:metabolism*; D011169:Portal Vein; D013004:Somatostatin / Q000031:analogs & derivatives* / Q000627:therapeutic use; D013964:Thyroid Neoplasms / Q000150:complications* / Q000473:pathology
D010455:Peptides; D013004:Somatostatin; D002116:Calcitonin; D015282:Octreotide