Medicinal therapy of gastroenteropancreatic neuroendocrine neoplasms

Neuroendocrine neoplasms (NEN) of the digestive tract represent a rare and heterogeneous group of malignancies with various clinical presentations and prognoses, thus complicating standardized therapeutic recommendations. Summary of current clinical recommendations for the medicinal treatment of gastrointestinal NEN. Discussion of published clinical studies and expert recommendations related to the medicinal therapy of gastroenteropancreatic NEN (GEP-NEN). The primary therapeutic goal in GEP-NEN treatment is complete surgical tumor resection. If this is not possible, treatment is usually carried out in a multimodal and multidisciplinary concept, taking the respective tumor entity, the individual tumor biology and the clinical symptoms into account. In the case of a distinct clinical hormonal syndrome, long-acting somatostatin analogues (SSA) are the mainstay of symptomatic therapy. They also represent the foundations for the antiproliferative treatment of well-differentiated, slow growing GEP-NET. Systemic peptide receptor radionuclide therapy (PRRT) with Lu(177)DOTATATE is an effective therapy in GEP-NET with adequate somatostatin receptor expression. For poorly differentiated neuroendocrine carcinomas (NEC) and pancreatic NET, systemic chemotherapy with platinum or streptozotocin-based regimens shows good results. The multi-targeted tyrosine kinase inhibitor SunitinibA (R) is approved for pancreatic NET and the mTOR inhibitor EverolimusA (R) for pancreatic and gastrointestinal NET. The therapeutic landscape of GEP-NETs has evolved significantly in recent years; however, navigating the current therapeutic algorithm may be challenging. The determination of a specific therapeutic concept should be made on an individual basis after discussion of all therapeutic options in a multidisciplinary team of NET specialists.