Neuroendocrine gastroenteropancreatic tumors are rather rare neoplasms with
an incidence of 1-2 cases per 100,000 people. They show rather varying tum
or biology and present sometimes distinct clinical symptoms such as hushing
, diarrhoea, hypoglycemia and gastric ulcers. The biochemical diagnosis is
today significantly improved by the introduction of chromogranin A as a gen
eral tumor marker,which is also useful in histopathology. Today the localiz
ation procedures include somatostatin receptor scintigraphy as the primary
investigation together with CT or ultrasonography. The basis for treatment
of neuroendocrine GEP tumors is not only a curative intent but merely ameli
oration of clinical symptoms, abrogation of tumor growth, maintaining and i
mprovement of quality of life. surgery has always to be considered in the t
reatment of neuroendocrine GEP tumors. It can be performed whenever during
the course of the disease but it may be more productive in earlier stages.
Liver dearterialization procedures can furthemore reduce the tumor masses i
n liver together with laser treatment or radiofrequency therapy. The medica
l treatment includes cytotoxic agents, alpha interferons and somatostatin a
nalogues. Somatostatin analogues will always be combined with the other two
alternatives to reduce clinical symptoms. Chemotherapy is particularly use
ful for patients with more aggressive tumors with high proliferation capaci
ty, whereas alpha interferon Is beneficial in classical midgut carcinoids w
ith low proliferation capacity. Quite recently somatostatin based radioacti
ve tumor targeted treatment has evolved with preliminar promising data but
further studies are needed to deliniate its future role in the treatment of
neuroendocrine tumors in patients.