A 46-year-old woman with jaundice was found to have enlargement of the enti
re pancreas on computed tomography. Endoscopy revealed a tumor at the papil
la of Vater, although examination of biopsy specimens did not demonstrate a
malignancy. The jaundice resolved spontaneously, but reappeared at 7 month
s. Also noted was increased swelling of the papilla. The serum pancreatic e
ndocrine and exocrine hormone concentrations were within the reference rang
es. At surgery, a hard mass was found occupying the entire pancreas. Examin
ation of incisional biopsy specimens from the pancreatic mass and papilla o
f Vater revealed an islet cell tumor. A total pancreatectomy was performed.
The tumor extended from the pancreatic tail to the papilla of Vater. Histo
pathologic and immunopathologic examination revealed a nonfunctional islet
cell carcinoma. There was no evidence of recurrence at 3 years. A good outc
ome can be achieved with resection of non-functioning islet cell carcinomas
, even if they are advanced.