Zusammenfassung
Hintergrund
Neuroendokrine Neoplasien (NEN) des Verdauungstrakts stellen eine seltene und heterogene Gruppe von Tumorerkrankungen dar. Dies erschwert die Vorgabe einheitlicher Therapieschemata.
Ziel
Die aktuelle Datenlage zur medikamentösen Therapie gastrointestinaler NEN soll dargestellt werden.
Methoden
Diskussion publizierter Studien und Expertenempfehlungen zur medikamentösen Therapie von gastroenteropankreatischen neuroendokrinen Neoplasien (GEP-NEN)
Ergebnisse
Wichtigstes therapeutisches Prinzip ist die komplette chirurgische Tumorentfernung. Ist diese nicht möglich, erfolgt die Therapie in der Regel multimodal und multidisziplinär und orientiert sich an der Tumorentität, dem individuellen Spontanverlauf sowie der Beschwerdesymptomatik. Bei Vorliegen eines spezifischen klinischen Hormonsyndroms sind langwirksame Somatostatinanaloga (SSA) die Therapie der Wahl. Sie stellen auch die Grundlage der antiproliferativen Therapie gut differenzierter gastroenteropankreatischer neuroendokriner Tumoren (GEP-NET) dar. Bei ausreichender Somatostatinrezeptor-Expression des Tumors steht als systemische Therapieoption die Peptidradiorezeptortherapie (PRRT) zur Verfügung. Bei gering differenzierten neuroendokrinen Karzinomen und NET des Pankreas stellen Platin- bzw. Streptozotocin-basierte Chemotherapien eine wichtige Therapieoption dar. Der Multityrosinkinaseinhibitor Sunitinib und der mTOR-Inhibitor Everolimus sind für die Behandlung pankreatischer NET zugelassen, Everolimus zudem auch für gastrointestinale NEN.
Schlussfolgerungen
Zur Behandlung von GEP-NEN stehen eine Reihe effektiver therapeutischer Optionen zur Verfügung. Die Entscheidung für ein Therapiekonzept sollte individuell im Rahmen eines in der Behandlung dieser Tumoren erfahrenen interdisziplinären Tumorboards festgelegt werden.
Abstract
Background
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.
Objective
Summary of current clinical recommendations for the medicinal treatment of gastrointestinal NEN.
Methods
Discussion of published clinical studies and expert recommendations related to the medicinal therapy of gastroenteropancreatic NEN (GEP-NEN).
Results
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 Lu177DOTATATE 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 Sunitinib® is approved for pancreatic NET and the mTOR inhibitor Everolimus® for pancreatic and gastrointestinal NET.
Conclusion
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.
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Die Autoren weisen auf folgende Interessenskonflikte hin: C. Fottner: Vortragshonorare/Kongressreisekosten-Unterstützung durch die Firmen Novartis Oncology GmbH, Ipsen Pharma GmbH, Pfizer Oncology. M. M. Weber: Vortragshonorare/Kongressreisekosten-Unterstützung durch Novartis Oncology GmbH, Ipsen Pharma GmbH, Pfizer Oncology und Forschungsförderung durch Novartis und Ipsen.
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Fottner, C., Weber, M.M. Medikamentöse Therapie neuroendokriner Neoplasien des Gastrointestinaltrakts. Onkologe 24, 124–132 (2018). https://doi.org/10.1007/s00761-017-0310-y
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DOI: https://doi.org/10.1007/s00761-017-0310-y
Schlüsselwörter
- Neuroendokrine Neoplasie
- Neuroendokriner Tumor
- Somatostatinanaloga
- Gastroenteropankreatische NEN
- Peptidradiorezeptortherapie