Endocrine pancreatic tumors (EPTs) are rare but have a remarkably better pr
ognosis than adenocarcinoma of the pancreas. Patients with EPTs benefit fro
m surgical and medical therapy, which may alleviate symptoms due to hormona
l excess and increase survival. Patients with large or malignant EPTs with
infiltrative disease may suffer from local complications, including gastroi
ntestinal bleeding and obstruction and involvement of the superior mesenter
ic (SMV) and portal (PV) veins. Among 31 patients with operable and large o
r malignant EPTs, 7 had hormone-producing syndromes (insulin, glucagon), an
d 24 had clinically nonfunctioning EPTs. Surgery in these patients included
vascular reconstruction of the SMV/PV (n = 4), resection of infiltrated ad
jacent organs (n = 5; stomach, transverse colon), or resection of concomita
nt liver metastases (n = 3). Four patients with conspicuously large insulin
omas, and three with glucagonoma were successfully operated on with allevia
tion of hormonal symptoms. Among the 24 nonfunctioning EPTs, 5 patients had
been explored earlier and their tumors judged inoperable due to locally in
vasive disease or misdiagnosis as pancreatic adenocarcinoma. The operations
were performed with no mortality and low morbidity. We conclude that large
and malignant EPTs with limited spread of disease may benefit from a combi
nation of medical and surgical therapy.