Neuroendokrine Neoplasien (NEN) sind klinisch und prognostisch extrem heterogene Tumorerkrankungen. Bei begrenzter Evidenz für einzelne Entitäten sollte die meist multimodale Therapie in einem erfahrenen interdisziplinären Team festgelegt werden. Dieser Beitrag stellt die Grundzüge der Systemtherapie bei metastasierter Erkrankung dar. Dabei werden Standardtherapien, wichtige Kriterien der Therapieauswahl sowie Neuentwicklungen beleuchtet.
Abstract
Due to the complexity and heterogeneity of metastatic NEN an interdisciplinary expert team should be involved in an individualized treatment strategy. SSA is the mainstay of antisecretory treatment in most functioning tumors. In antiproliferative intention SSA are first line treatment in receptor positive low proliferative NET. In intestinal metastatic disease PRRT is best established second line treatment. Further options are Everolimus (labeled) and tyrosine kinase inhibitors (off-label). Everolimus is the only approved drug for antiproliferative treatment in patients with metastatic lung NET, whereas in pancreatic NET more therapeutic options are available (SSA, chemotherapy, PRRT, Sunitinib, Everolimus) without a standard of best sequence. In patients with metastatic NEC standard first line treatment (platinum + etoposide) has not changed for decades and new treatment options for this fatal disease are urgently needed. Benefit of immunotherapy is limited to a small subset of patients – new combinations are under investigation. This review summarizes the standard of care, criteria of treatment selection and new developments for systemic therapy in patients with metastatic NEN.
Schlüsselwörter
neuroendokrine Neoplasie - neuroendokriner Tumor - neuroendokrines Karzinom - Somatostatinanaloga - Chemotherapie
Keywords
neuroendocrine neoplasia - neuroendocrine tumor - neuroendocrine carcinoma - somatostatin analogs - chemotherapy
