The efficacy of chemotherapy in digestive neuroendocrine tumors (NET) depen
ds on primary site and histological differentiation. Many reports have sugg
ested a superior activity of chemotherapy for pancreatic NET than for metas
tatic carcinoid tumors with response rates ranging from 40 to 60% compared
to 20%. The standard chemotherapy for pancreatic NET is a combination of ad
riamycin and streptozocin and to a lesser extent a combination of 5FU and s
treptozocin. In contrast, there is no clear standard chemotherapy for carci
noid tumors and if most oncologists use a combination of 5FU and streptozoc
in in the case of advanced, progressive and nonresectable carcinoid tumors,
the results are mostly poor and the benefit seldom counterbalances its tox
icity. In these carcinoid tumors the combination of hepatic artery ischemia
alternating with chemotherapy has given impressive results in one study, w
hich, however, have never been confirmed. Tumor cell differentiation is a m
ajor prognostic factor and some reports have suggested a higher chemosensit
ivity for undifferentiated or poorly differentiated NET with tumor response
rates ranging from 41 to 69% when a VP16-CDDP combination is used. This ch
emosensitivity is, unfortunately, as in small cell lung carcinomas, of shor
t duration. Related to this special problem and the number of other active
treatments in NET, the place of chemotherapy always has to be discussed in
a multidisciplinary fashion. Surgical excision, chemoembolization, interfer
ons and somatostatin analogues have to be emphasized and eventually combine
d with chemotherapy, especially in slowly growing tumors. New active chemot
herapy regimens have to be tested clearly in this orphan group of tumors wh
ich does not hold much interest to the pharmaceutical companies. Copyright
(C) 2000 S. Karger AG, Basel.