Neuroendocrine tumors of the gastroenteropancreatic system present not
only a diagnostic but also a therapeutic challenge. In case of limite
d neuroendocrine tumor disease curative surgery is the goal to aim for
. But in patients with metastatic disease (palliative) surgery may be
indicated, too. (Chemo-) Embolization should to be considered for live
r metastases causing severe local symptoms. Recently, biotherapy with
somatostatins or interferon-alpha has revolutionized the treatment of
metastatic neuroendocrine tumor disease of the gastroenteropancreatic
system. Both somatostatins and interferon-alpha are mostly effective i
n controlling the carcinoid syndrome; they lead to significant tumor r
eductions at least in some cases and thereby improve the quality of li
fe and possibly lengthen survival. For patients with anaplastic neuroe
ndocrine tumors or with advanced neuroendocrine tumor disease of the p
ancreas, chemotherapy may still be the first-line treatment modality;
the response rate is 40-70%. Present clinical trials investigate the e
fficacies of combinations of different biotherapies.