Gastrointestinal
neuroendocrine tumors are slowly growing and
metastases are often limited to the liver. As a result of their favorable
biological behavior these
tumors have a relatively good prognosis even in metastatic stage. Due to a variety of therapeutic options patients with malignant
neuroendocrine tumors may survive for extended periods of time up to ten years. Often a combination of different treatments and also alternation between the different therapeutic regimes is needed. A patient with excessive
WDHA-syndrome and severe metabolic disturbances due to a
pancreatic VIPoma with metastatic spread into the liver and abundant hormonal secretion is presented.
Cytotoxic agents (
streptozocin,
5-fluorouracil and
adriamycin) were able to alleviate clinical symptoms and to control
tumor growth for six years. Analogues of
somatostatin (
octreotide) and
interferon alpha had been very useful in controlling clinical symptoms and
tumor progress for 18 months.
Cytotoxic agents or
octreotide were not able, however, to achieve any permanent cure. Eventually, treatment failure occurred with dramatic progression of symptoms and
tumor growth, unresponsive to any medical
therapy. Consequently, total
hepatectomy and
liver transplantation together with extirpation of the pancreatic primary
tumor was performed and succeeded in providing a normal life to the patient. In our opinion the overall outcome of patients with metastatic
VIPoma may be improved best by maintaining the patients on medical
therapy until treatment failure occurs. In case of extended hepatic
metastases orthotopic
liver transplantation might be considered for patients with symptomatic disease who no longer respond to conventional treatment modalities.