Metastatic neuroendocrine neoplasms

The clinical presentation of neuroendocrine neoplasms (NEN) varies depending on the site of origin, functional activity, histological differentiation and tumor growth behavior. Therapeutic strategies aim to improve the symptoms of clinical syndromes related to the secretion of amines and peptides, inhibit tumor growth and prevent complications. Novel molecular targeted drug therapies offer new treatment options in addition to established therapies, such as somatostatin analogs and chemotherapy. The precise positioning of these drugs and of peptide receptor radionuclide therapy still needs to be defined. In general, the different medical treatments are used in a sequential way and are in part combined with surgical and loco-regional/ablative procedures in a highly individualized approach. In certain clinical conditions, however, established treatment strategies are followed. This review presents the management of patients with small intestinal and pancreatic NEN with respect to the functionality and tumor grading. The use of novel targeted drug therapy is included in a therapeutic algorithm.