Background: Tumors of the neuroendocrine system are characterized by a
mine precursor uptake and decarboxylation, and they represent a hetero
geneous group of carcinomas including carcinoids, islet cell carcinoma
s of the pancreas, medullary thyroid carcinomas and Merkel cell carcin
omas. Their similar cytochemical and ultrastructural properties sustai
n the hypothesis of a common embryologic origin within the neural cres
t. Many of these tumors grow slowly, and reducing tumor burden represe
nts the treatment of choice. However, when surgery is not feasible, me
dical treatment has to be considered. Therapeutic approaches in metast
atic disease often do not consider the different biologic behaviors of
these neoplasms. Moreover, efficacy of the treatment is associated wi
th lack of a clear definition of the type of response: objective, symp
tomatic or biochemical. Methods: In this review we have analyzed the d
ifferent medical approaches used in the treatment of neuroendocrine tu
mors in an attempt to define their precise role in the different neopl
asms. Results: In carcinoid tumors, immunotherapy and the somatostatin
analogue can be efficaciously used for the control of carcinoid syndr
ome. For inhibition of tumor growth, chemotherapy should be used only
in patients with rapidly progressive disease, and the results are stil
l unsatisfactory. Conclusions: Although all these tumors appear to hav
e similar cytochemical properties, the responsiveness of the various n
eoplasms is very different. In the future, a specific treatment modali
ty and a clear definition of the type of response (objective, symptoma
tic or biochemical) need to be defined for each type of neuroendocrine
tumor.