Background. Duodenal somatostatinomas are rare tumors that are often a
symptomatic or present with local symptoms rather than with evidence o
f excess somatostatin production. To characterize the clinical present
ation and management of these neoplasms we reviewed the course of four
patients. Methods. The records of three men and one woman with duoden
al somatostatinomas, who were treated between 1988 and 1992, are revie
wed. Results. Three of the patients presented with vague gastrointesti
nal complaints, and one was free of symptoms. No patient had symptoms
of somatostatinoma syndrome, nor did any have associated neurofibromat
osis. 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 tumo
r and liver metastases underwent a Whipple's operation and resection o
f the liver lesions. Immunohistochemically all tumors predominantly co
ntained 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 b
e locally excised. Intraarterial methylene blue injection may help loc
alize very small tumors during operation. Larger tumors, including tho
se with localized metastases, should also be resected when possible.