Background: Duodenal somatostatinomas are rare tumors that are often asymptomatic or present with local symptoms rather than with evidence of excess somatostatin production. To characterize the clinical presentation and management of these neoplasms we reviewed the course of four patients.
Methods: The records of three men and one woman with duodenal somatostatinomas, who were treated between 1988 and 1992, are reviewed.
Results: Three of the patients presented with vague gastrointestinal complaints, and one was free of symptoms. No patient had symptoms of somatostatinoma syndrome, nor did any have associated neurofibromatosis. Three patients were diagnosed after biopsy of a submucosal mass during upper endoscopy. Three patients with tumors that measured less than 2 x 2 cm underwent local resection. One patient with a large tumor and liver metastases underwent a Whipple's operation and resection of the liver lesions. Immunohistochemically all tumors predominantly contained somatostatin, although each had traces of other neuropeptides. All tumors contained psammoma bodies. Follow-up periods ranged from 1 to 4 years.
Conclusions: Duodenal somatostatinomas often appear with only local symptoms or are completely asymptomatic. Small tumors may be locally excised. Intraarterial methylene blue injection may help localize very small tumors during operation. Larger tumors, including those with localized metastases, should also be resected when possible.