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Successful Treatment with SMS 201-995 of Cushing’s Syndrome Caused by Ectopic Adrenocorticotropin Secretion From a Metastatic Gastrin-Secreting Pancreatic Islet Cell Carcinoma

Departments of Medicine, Surgery, and Radiology, Erasmus University, Rotterdam The Netherlands

Address all correspondence and requests for reprints to: Steven W. J. Lamberts, Department of Medicine, University Hospital Dijkzigt, 40 Dr. Molewaterplein, 3015 GD Rotterdam, The Netherlands.

Signs and symptoms of Cushing’s syndrome developed rapidly after total gastrectomy in a 37-yr-old man with a metastatic gastrin-secreting islet cell carcinoma. Argyrophilic tumor cells in a lymph node removed during operation immunostained for gastrin and ACTH. Treatment for more than 6 months with the somatostatin analog SMS 201-995 (300 µ/day) greatly reduced serum gastrin levels and normalized plasma ACTH and cortisol levels and urinary cortisol excretion, and the signs and symptoms of Cushing’s syndrome disappeared. The size of the primary tumor in the head of the pancreas, which had grown rapidly before SMS 201-995 therapy, stabilized after 6 months of treatment with the analog. We conclude that SMS 201-995 can reduce ACTH as well as gastrin secretion from islet cell carcinomas as well as control tumor growth.

Received April 5, 1988.

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