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Dtsch Med Wochenschr 2024; 149(15): 879-886
DOI: 10.1055/a-2173-0588
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Systemtherapie von neuroendokrinen Neoplasien

Systemic treatment of patients with metastatic neuroendocrine Neoplasia
Anja Rinke
,
Friederike Eilsberger
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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.

Kernaussagen

  • Metastasierte neuroendokrine Neoplasien sind extrem heterogene und komplexe Krankheitsbilder, die einer individualisierten und interdisziplinär festgelegten Behandlungsstrategie bedürfen.

  • Somatostatin-Analoga sind bei niedrig proliferativen rezeptorpositiven NET verschiedener Primär-Tumorlokalisationen eine geeignete Erstlinientherapie, zudem bei hormonaktiven Tumoren Basis einer antisekretorischen Therapie.

  • In der intestinalen Situation ist die PRRT meist die Zweitlinientherapie, Everolimus eine weitere Systemoption.

  • Bei pankreatischen NET mit hoher Tumorlast, hoher Proliferationsrate und/oder hoher beobachteter Dynamik stellt die Chemotherapie mit Streptozotocin + 5-FU oder Capecitabin + Temozolomid eine wichtige Therapieform dar.

  • Everolimus und Sunitinib sind zugelassene Behandlungen bei metastasierten pankreatischen NET; neuere TKI wie Cabozantinib könnten bei kürzlich positiven Phase-III-Daten eine neue Therapieoption werden.

  • Platin + Etoposid bleibt Standard in der Erstlinie bei metastasiertem NEC.

  • Die besten Zweitliniendaten bei NEC liegen für FOLFIRI vor, neue Optionen bei weiter ungenügender Effektivität werden dringend benötigt.

  • Eine frühe molekulare Untersuchung der Tumorprobe bei NEC sollte zur Detektion einer möglichen zielgerichteten Therapiealternative erfolgen.

Schlüsselwörter

neuroendokrine Neoplasie - neuroendokriner Tumor - neuroendokrines Karzinom - Somatostatinanaloga - Chemotherapie

Keywords

neuroendocrine neoplasia - neuroendocrine tumor - neuroendocrine carcinoma - somatostatin analogs - chemotherapy


Publication History

Article published online:
16 July 2024

© 2024. Thieme. All rights reserved.

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