Endocrine tumours of the gastrointestinal tract and pancreas may present
at different disease stages with either hormonal or hormone-related
symptoms/syndromes, or without hormonal symptoms. They may occur either
sporadically or as part of hereditary syndromes. In the therapeutic
approach to a patient with these tumours, excessive hormonal secretion
and/or its effects should always be controlled first. Tumour-related
deficiencies or disorders should also be corrected. Subsequently, control
should be aimed at the tumour growth. Surgery is generally considered as
first-line therapy for patients with localized disease, as it can be
curative. However, in patients with metastatic disease the role of
first-line surgery is not clearly established and other therapies should
be considered, such as non-surgical cytoreductive therapies, biotherapy
(with somatostatin analogues or interferon-alpha), embolization and
chemoembolization of liver metastases, chemotherapy (with single or
multiple dose regimens) and peptide receptor-targeted radiotherapy. The
delicate balance of the use of the different therapeutical options in
patients with endocrine tumours of the gastrointestinal tract and pancreas
emphasizes the importance of team approach and team expertise
