Somatostatinoma of the papilla major and minor associated with gastrointestinal stromal tumor (GIST)

Abstract

Somatostatinomas are very rare neuroendocrine tumors that have imunohistochemicpositivity for somatostatin. The incidence of these tumors is higher in patients withthe Von Recklinghausen’s syndrome. There are four cases in the literature that show coexistencebetween somatostatinoma and gastrointestinal stromal tumors (GIST), all patients withneurofi bromatosis. In this report we describe a non syndromic patient who had two primarysomatostatinomas of both major and minor papillae, and nine jejunal GIST nodules. The diagnosiswas confi rmed by the anatomopathology and imunohistochemistry, and the treatmentwas duodenopancreatectomy and resection of the jejunal nodules. After the operation, thepatient developed delayed gastric emptying. She was released from the Hospital in the 34th day,with oral feeding reestablished. This is the fi rst case reported in the literature that describes asynchronic appearance of these tumors in a patient that does not have neurofi bromatosis.