Metastatic jejunal VIPoma: Beneficial effect of combination therapy with interferon-alpha and 5-fluorouracil

The VIPoma syndrome is rare. It is usually caused by a neuroendocrine tumor located in the pancreas. Somatostatin analogs and interferon-cr can be hel pful in the symptomatic control of the disease, but the efficacy of chemoth erapy in metastatic disease is limited. We report the case of a 32-yr-old p atient who had a primary intestinal VIPoma with peritoneal carcinomatosis a nd hepatic metastases. Somatostatin analogs and conventional chemotherapy r egimens were not effective on VIPoma syndrome and tumor progression. The co mbination of 5- fluorouracil and interferon-a was associated with a major c linical improvement and tumor regression. Further investigations should eva luate the place of such a combination as a first line treatment for patient s with metastatic neuroendocrine tumors. (Am J Gastroenterol 2000; 95:289-2 93. (C) 2000 by Am. Coll. of Gastroenterology).