Multimodal treatment of neuroendocrine tumors of the pancreas and the ampulla of Vater

Neuroendocrine tumors are not seen frequently. They are most commonly located in the small bowel including the vermiform appendix. Neuroendocrine tumors of pancreatic origin are extremely rare. Symptoms caused by excessive hormone production by large liver metastases often lead to their diagnosis. Preoperative diagnostics include analysis of specific hormones in serum and urine, ultrasound, CT and somatostatin receptor scintigraphy. Liver metastases of neuroendocrine tumors of the pancreas are common at the time of diagnosis. Curative resection should be performed whenever possible, although patients often benefit from debulking procedures, too. Liver metastases can be subjected to surgical resection, embolization, regional chemotherapy or local procedures such as alcohol injection or cryoablation. As an exception, liver transplantation can be considered in selected cases where radical surgery for the primary tumor could be performed and extrahepatic metastases are not present. Supplementary or alternative options include octreotide and/or interferon-a administration. In this article, we report on 6 patients suffering from primary neuroendocrine tumors of the pancreas or the ampulla of Vater who were treated at our department over the past 5 years. In addition, we discuss our own experience with this rare condition in the light of the recent literature.