Tumors arising from the pancreatic islet cells are rare and represent a het
erogeneous group of benign or malignant lesions. Most tumors present with w
ell characterized syndromes, whereas others appear to be nonfunctioning. Th
e clinical features of 11 men and 7 women with nonfunctioning islet cell ca
rcinomas operated on between 1983 and 1998 were reviewed. The median patien
t age was 53.5 years (range 26-74 years). The most frequent presenting symp
toms were abdominal pain (13 patients), weight loss (7 patients), and obstr
uctive jaundice (4 patients). Gut hormone profiles were normal in all patie
nts. Abdominal sonography and computed tomography localized the tumor in 17
patients, and correct prediction of an endocrine tumor was achieved in 12
patients. Six of seven patients showed a hypervascular tumor upon angiograp
y, and seven of eight patients preoperatively had positive somatostatin rec
eptor scintigraphy. At operation, regional or distant metastases were prese
nt in 15 (83%) and 6 (33%) patients, respectively. Eleven patients underwen
t potentially curative resections, and the remaining seven patients were ma
naged palliatively by resection (four patients) or bypass procedures (three
patients). Three patients had up to three more resection for metastases. E
ight patients received postoperative octreotide, interferon a: therapy, or
both. The overall cumulative 5- and 10-year survival rates were 65.4% and 4
9.1%, respectively. Of the II patients who underwent curative resection, 10
were alive after a median follow-up of 63 months (range 7-180 months), but
only 5 are free from disease. Although surgical cure is rare in nonfunctio
ning islet cell carcinomas, significant long-term palliation can be achieve
d in a large proportion of patients with an aggressive surgical approach an
d, when indicated, additional medical therapy.