Neuroendocrine tumors (NETs) represent a very heterogenous group of neoplasms, comprising approximately 2% of all malignant tumors of gastroenteropancreatic system. Although they are characterized by a relatively slow tumor growth, they have a malignant potential and, in fact, most of them are diagnosed when distant metastases have been developed. NETs, when functioning, are causing symptoms related not only to the tumour mass itself, but also to the specific hormonal peptide that they secrete. The major goals of treatment in patients with NETs are : A) Medical control of patients symptoms B) Resection of tumor primary and if possible, metastatic lesions and, C) Control of tumor growth in cases of advanced disease. NETs symptoms associated with hormonal hypersecretion may not only impair the patients quality of life, but also may be life-threatening. The greatest impact for control of symptoms has been afforded by the somatostatin analogues. In NETs, surgery remains the first-line treatment in patients with localized disease at presentation, as it can be curative. The optimal surgical management depends on tumor type, location and size. In the presence of liver metastases, curative liver resection is possible in approximately 10% of patients with solitary lesions or lesion confined to one lobe. In advanced NETs when surgery is not feasible, several treatment options are available such as long acting somatostatin analogues, interferon, radionuclide peptide treatment, transarterial hepatic (chemo)-embolization, systemic chemotherapy and recently molecular targeted treatment with new agents such as sunitinib and everolimus. These treatments, although cannot achieve significant radiological partial response, they are associated with increased progression-free survival. The optimal management of patients with NETs involves the coordination of several specialties. A multidisciplinary approach by gastoenterologists, oncologists, endocrinologists, radiologists, nuclear medicine physicians, histopathologists and surgeons is required in order to achieve a more individualized therapeutic approach for each patient.
Declaration of interest: There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported.
Funding: No specific grant from any funding agency in the public, commercial or not-for-profit sector.