ROLE OF SURGERY IN MANAGEMENT OF ADRENOCORTICOTROPIC HORMONE-PRODUCING ISLET-CELL TUMORS OF THE PANCREAS

Background. Ectopic adrenocorticotropic hormone-producing islet cell t umors of the pancreas (ACTH-ICT) are a rare cause of Cushing's syndrom e with a severe and rapidly progressive clinical course. Methods. Char ts were reviewed on all patients evaluated and treated for proven Cush ing's syndrome caused by ACTH-ICT (n = 12), specifically for the role of surgery in the management of this disease. Results. Ten (83%) of tw elve patients with ACTH-ICT had liver metastases at the time of diagno sis (eight of eight with Zollinger-Ellison syndrome, two of four witho ut Zollinger-Ellison syndrome). Surgical management of the primary tum or included three patients who underwent distal pancreatectomy combine d with hepatic resection and one patient who underwent laparoscopic en ucleation of a tumor from the pancreatic tail. Eight of twelve patient s underwent bilateral adrenalectomy to control symptoms of Cushing's s yndrome, including three patients who underwent concurrent distal panc reatectomy and hepatic resection. Six of twelve patients died of the d isease within 21/2 years of diagnosis, four are alive with progressive hepatic metastases, and one has biochemical evidence of disease. Conc lusions. ACTH-ICT of the pancreas is an aggressive tumor, particularly when there is coproduction of gastrin. The benefit of aggressive surg ical resection of primary or metastatic ACTH-ICT has not been establis hed. However, palliative bilateral adrenalectomy is justified, because no patients had biochemical cures after aggressive surgical resection in this series.