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Medical treatment of endocrine gastroenteropancreatic tumors.

Neuroendocrine gastroenteropancreatic (GEP) tumors are rather rare neoplasms with an incidence of 1-2 cases per 100,000 people [1, 2, 3, 4]. They originate from any of the various cell types belonging to the neuroendocrine system. A general characteristic of GEP endocrine tumors is that the vast majority produce and secrete a multitude of peptide hormones and amines. Several syndromes can be associated with GEP endocrine tumors, caused by hyperproduction of a specific hormone, and usually liver metastases are pre sent in patients because of the malignancy of the tumors [5, 6, 7, 8, 9, 10]. The syndromes include: carcinoid syndrome [10], ZollingerEllison syndrome [6], the so-called "insulinoma syndrome" [5], "glucagonoma syndrome" [7], Verner-Morrison syndrome, which is brought about by high circulating levels of vasointestinal peptide (VIP) [8], and finally the "somatostatinoma syndrome" [9]. Otherwise, there are some endocrine tumors, usually located in the pancreas, that are not associated with signs or symptoms of hormone hypersecretion, and therefore they are called nonfunctioning. Because of the rarity of these types of tumors, their possible episodic expression and the variable clinical symptoms, the patients are often diagnosed late in the advanced stages of the disease. In contrast to other metastasized tumors, the patients with gut and pancreatic neoplasms often survive for long periods due to the slow tumor progression; instances of survival greater than 10 years have been reported [11]. For these reasons, the patients with advanced metastatic disease should be treated aggressively with medical and surgical therapies aimed at reducing both symptoms and complications through strategies that reduce tumor bulk and block hormonal effects. Neuroendocrine tumor treatment is aimed at reducing the tumor mass and inhibiting hormonal release. Therefore, a multimodal approach is necessary where different therapeutic means can be used synchronously or metachronously [12].