Neuroendocrine neoplasms of the digestive tract

Neuroendocrine neoplasms (NEN) of the digestive tract are heterogeneous diseases with variable clinical courses. They account for approximately 1% of all malignancies. Due to hormone-excess syndromes, functionally active NEN require dedicated diagnostic strategies and treatment. The only curative treatment of NEN is complete excision. Further options include somatostatin analogs, molecular targeted treatment, peptide receptor radionuclide therapy, and systemic chemotherapy. The number of phase III trials is limited and comparative studies are lacking in most situations. Therefore, an established treatment algorithm is only rarely available, and measures are implemented in an individualized manner. The treatment target for advanced differentiated neuroendocrine tumors is growth stabilization. The objective response rate of undifferentiated neuroendocrine carcinomas to systemic chemotherapy is higher, but statistically of short duration. © 2020, Springer Medizin Verlag GmbH, ein Teil von Springer Nature.