Neuroendocrine tumors are not seen frequently. They are most commonly locat
ed in the small bowel including the vermiform appendix. Neuroendocrine tumo
rs of pancreatic origin are extremely rare. Symptoms caused by excessive ho
rmone production by large liver metastases often lead to their diagnosis. P
reoperative diagnostics include analysis of specific hormones in serum and
urine, ultrasound, CT and somatostatin receptor scintigraphy. Liver metasta
ses of neuroendocrine tumors of the pancreas are common at the time of diag
nosis. Curative resection should be performed whenever possible, although p
atients 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 s
urgery for the primary tumor could be performed and extrahepatic metastases
are not present. Supplementary or alternative options include octreotide a
nd/or interferon-a administration. In this article, we report on 6 patients
suffering from primary neuroendocrine tumors of the pancreas or the ampull
a of Vater who were treated at our department over the past 5 years. In add
ition, we discuss our own experience with this rare condition in the light
of the recent literature.