METASTATIC PANCREATIC VIPOMA - DETERIORATING CLINICAL COURSE AND SUCCESSFUL TREATMENT BY LIVER-TRANSPLANTATION

Gastrointestinal neuroendocrine tumors are slowly growing and metastas es are often limited to the liver. As a result of their favorable biol ogical behavior these tumors have a relatively good prognosis even in metastatic stage. Due to a variety of therapeutic options patients wit h malignant neuroendocrine endocrine tumors may survive for extended p eriods of time up to ten years. Often a combination of different treat treatments and also alternation between the different therapeutic reg imes is needed. A patient with excessive WDHA-syndrome and severe meta bolic disturbances due to a pancreatic VIPoma with metastatic spread i nto the liver and abundant hormonal secretion is presented. Cytotoxic agents (streptozocin. 5-fluorouracil and adriamycin) were able to alle viate clinical symptoms and to control tumor growth for six years. Ana logues of somatostatin (octreotide) and interferon alpha had been very useful in controlling clinical symptoms and tumor progress for 18 mon ths. Cytotoxic agents or octreotide were not able, however, to achieve any permanent cure. Eventually, treatment failure occurred with drama tic progression of symptoms and tumor growth. unresponsive to any medi cal therapy. Consequently, total hepatectomy and liver transplantation together with extirpation of the pancreatic primary tumor was perform ed and succeeded in providing a normal life to the patient In our opin ion the overall outcome of patients with metastatic VIPoma may be impr oved best by maintaining the patients on medical therapy until treatme nt failure occurs. In case of extended hepatic metastases orthotopic l iver transplantation might be considered for patients with symptomatic disease who no longer respond to conventional treatment modalities.