Update on cholangiocarcinoma: potential impact of genomic studies on clinical management

Abstract

The term cholangiocarcinoma (CCA) comprises neoplasms of the intrahepatic, perihilar, and distal bile duct. Five-year survival rates of patients with CCA are below 20 %, and no targeted therapy could prove a benefit in comparison to the standard treatment of cisplatinum and gemcitabine. In recent years, next generation sequencing studies revealed a profound genomic heterogeneity of CCA subtypes potentially affecting the design of future therapy trials. This review provides a concise update on current clinical management of CCA including data of recent genomic studies and differences between CCA subtypes.

Zusammenfassung

Die Entität Cholangiokarzinom (CCA) beinhaltet Neoplasien aus dem intrahepatischen, perihilären und distalen Gallengang. Weniger als 20 % der Patienten mit einem CCA überleben länger als 5 Jahre und bislang konnte keine zielgerichtete Therapie einen Überlebensvorteil gegenüber dem Standard aus Cisplatin und Gemcitabine zeigen. In den letzten Jahren haben Next-Generation-Sequencing Daten beim CCA und seinen Subtypen eine ausgeprägte genetische Heterogenität offenbart, welche die Planung zukünftiger Therapiestudien beeinflussen könnte. Diese Arbeit soll einen kompakten Überblick zur aktuellen Diagnostik und Therapie des CCA geben und in diesem Rahmen auch auf Daten aus Sequenzierungsstudien sowie auf die Unterschiede der CCA-Subtypen eingehen.

Schlüsselwörter

Cholangiokarzinom - Gallengangstrakt - Genetik - next generation sequencing - gezielte Therapien - immunmodulatorische Therapien

Key words

cholangiocarcinoma - biliary tract - genomic background - next generation sequencing - heterogeneity - targeted therapy - immune modulating therapy

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