Zusammenfassung
GIST sind mesenchymale Tumoren, deren Ursprung in den interstitiellen Cajal-Zellen vermutet wird. Charakteristisch ist eine CD117-Expression, die auf einer aktivierenden Mutation des Tyrosinkinaserezeptors basiert, die zu einem unkontrollierten Proliferationsreiz und dem Verlust der Apoptosefunktion mit konsekutivem Tumorwachstum führt. Einige GIST sind CD117-negativ und weisen eine Mutation des PDGFRA-Gens („platelet derived growth factor receptor α“) auf. Die Bestimmung des c-KIT - bzw. PDGFRA-Status ist deshalb für die Diagnosestellung obligat. Klinisch gibt es 3 entscheidende Prognosefaktoren: Tumorgröße, -lokalisation und -mitoserate. Goldstandard für lokalisierte, resezierbare GIST ist die komplette chirurgische Resektion. In Abhängigkeit vom Rezidivrisiko erfolgt eine Therapie mit Imatinib (adjuvant 400 mg über 3 Jahre). Auch bei lokal fortgeschrittenen, inoperablen oder metastasierten GIST ist Imatinib je nach Mutationsstatus aktuelle Standardtherapie.
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Höffken, N., Tannapfel, A., Reinacher-Schick, A. (2015). Diagnostik und Therapie von gastrointestinalen Stromatumoren (GIST). In: Adamietz, I., et al. Weiterbildung Onkologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-44835-9_15
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