Background. Nonfunctioning islet cell carcinoma of the pancreas has a
variable and often indolent natural history, which has resulted in a w
ide range of treatment recommendations. To more clearly define the nat
ural history and appropriate treatment of this disease, we reviewed ou
r institutional experience over the last 39 years. Methods. The record
s of all patients confirmed to have a nonfunctioning islet cell carcin
oma of the pancreas were retrospectively reviewed. Kaplan-Meier life t
ables were constructed and log-rank comparisons were performed. Result
s. The 73 patients studied had an overall 5-year actuarial survival ra
te of 50%. Patients with localized disease at presentation (n = 39) ha
d a significantly higher survival rate (p = 0.03) compared with patien
ts with metastatic disease (n = 34). The 79 patients who underwent a p
otentially curative resection of the primary tumor had a significantly
higher survival rate (p = 0.03) compared with the 20 patients with lo
cally advanced, unresectable, nonmetastatic disease. Nine of these 20
patients died of complications of the primary tumor. In contrast, only
2 of 22 cancer-related deaths in the 34 patients with metastatic dise
ase at diagnosis were due to the primary tumor. Conclusions. (1) Surgi
cal resection should be performed in patients with resectable nonmetas
tatic disease. (2) Resection of the primary tumor in the presence of m
etastatic disease is rarely indicated (3) Innovative treatment strateg
ies are needed for patients with locally advanced, unresectable, nonme
tastatic tumors of the pancreatic head.