Immuntherapie bei neuroendokrinen Tumoren

Abstract

BackgroundNeuroendocrine tumors (NET) of the digestive system and lung carcinoids are rare neoplasms. In advanced unresectable disease, various systemic therapies are used. Primary tumor site, pathological classification including grading, tumor slope and tumor burden, as well as expression of somatostatin receptors and functional activity have an impact on treatment choices. Established treatment options include somatostatin analogs (octreotide, lanreotide), peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTA-TATE, molecular targeted therapies (everolimus, sunitinib), and systemic chemotherapy with streptozotocin- or temozolomide-based regimes in well-differentiated NET, or platinum/etoposide in poorly differentiated neuroendocrine carcinoma. There is a high unmet need for novel therapies.AimReview of the current status of immunotherapy in neuroendocrine neoplasms of the digestive system and of lung carcinoids.ResultsImmunotherapy with checkpoint inhibitors is a well-established therapy for example in non-small cell lung cancer (NSCLC), melanoma, hepatocellular carcinoma, and Merkel cell carcinoma, and in combination therapy in small cell lung cancer (SCLC). Studies are limited so far and demonstrate absent or low response rates in well-differentiated NET G1/G2. Other subgroups of patients have shown benefit particularly with dual checkpoint inhibitor therapy with 17–44% objective response rates (ORR). These include primarily neuroendocrine carcinomas of gastroenteropancreatic origin, but also atypical lung carcinoids and possibly also more proliferative NET G3, although data for NET G3 are limited.