Resection of a non-functioning islet cell carcinoma occupying the entire pancreas

A 46-year-old woman with jaundice was found to have enlargement of the entire pancreas on computed tomography. Endoscopy revealed a tumor at the papilla of Vater, although examination of biopsy specimens did not demonstrate a malignancy. The jaundice resolved spontaneously, but reappeared at 7 months. Also noted was increased swelling of the papilla. The serum pancreatic endocrine and exocrine hormone concentrations were within the reference ranges. At surgery, a hard mass was found occupying the entire pancreas. Examination of incisional biopsy specimens from the pancreatic mass and papilla of Vater revealed an islet cell tumor. A total pancreatectomy was performed. The tumor extended from the pancreatic tail to the papilla of Vater. Histopathologic and immunopathologic examination revealed a non-functional islet cell carcinoma. There was no evidence of recurrence at 3 years. A good outcome can be achieved with resection of non-functioning islet cell carcinomas, even if they are advanced.