Zusammenfassung
Neuroendokrine Tumoren (NET) stellen eine heterogene Erkrankung mit unterschiedlicher klinischer Präsentation und Prognose dar. Die Auswahl der medikamentösen Therapie orientiert sich in erster Linie an der Funktionalität, der Proliferationsrate (Grading) und dem Wachstumsverhalten des Tumors. Daneben sind Primärtumorsitz und die Tumorausdehnung von Bedeutung. Zu den verfügbaren Therapieoptionen gehören Somatostatinanaloga, Interferon α, neue molekular-zielgerichtete Therapien (z. B. Sunitinib, Everolimus) und die systemische Chemotherapie. Spezielle Entitäten erfordern zusätzliche Therapien (z. B. Protonenpumpeninhibitoren beim Gastrinom). Da keine komparativen Studien vorliegen, kann keine Therapiesequenz definiert werden und die verfügbaren Therapien werden oft sequenziell und individualisiert eingesetzt. Selten werden bei NET G1/G2-Tumorremissionen erzielt, häufig jedoch Wachstumsstabilisierungen, die nach vorausgegangener Tumorprogression als Therapieerfolg angesehen werden können. Das objektive Ansprechen auf systemische Chemotherapien ist dagegen bei neuroendokrinen Karzinomen (G3) hoch, jedoch oft von kurzer Dauer. Es existieren keine etablierten Reservetherapien.
Abstract
Neuroendocrine tumors (NET) are a heterogeneous disease with distinct clinical presentation and prognosis depending on the site of origin and secretion of bioactive substances. The choice of the medical therapy for NET is based on the functionality, tumor grading and growth behaviour. Primary tumor site and tumor extent are other important parameters for decision making. Medical therapies include somatostatin analogs, interferon α, novel targeted therapies such as sunitinib and everolimus, and systemic chemotherapy. Special subtypes of NET require additional treatment, e.g. proton pump inhibitors in gastrinoma. In the absence of comparative trials there is no clear algorithm for antiproliferative treatment and available drugs are frequently used in a sequential and individualized manner. Objective tumor remissions are rarely achieved with medical therapies in neuroendocrine tumors G1/G2, but disease stabilisation may be considered a treatment success after prior tumor progression. Chemotherapy may induce partial tumor remissions in poorly differentiated NET (neuroendocrine carcinoma G3), but these are short lasting and there are no established second-line therapies.
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Die korrespondierende Autorin weist auf folgende Beziehungen hin: Prof. Dr. Pavel hat Honorare für Vorträge und Beratungstätigkeit (Advisory Board Meetings) von den Firmen Novartis, IPSEN und Pfizer erhalten sowie Studienunterstützung durch die Firma Novartis.
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Pavel, M. Medizinische Therapie und Chemotherapie von neuroendokrinen Tumoren. Onkologe 17, 592–601 (2011). https://doi.org/10.1007/s00761-011-2053-5
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DOI: https://doi.org/10.1007/s00761-011-2053-5