Gallengangs- und Gallenblasenkarzinome: auf dem Weg zur personalisierten Therapie

Was ist neu?

Nomenklatur: Überbegriff maligne biliäre Tumoren Unter dem Begriff maligne biliäre Tumoren (engl. biliary tract cancer) werden weiterhin Karzinome der intra- und extrahepatischen Gallengänge und der Gallenblase zusammengefasst, deren auch molekularpathologische Unterschiede immer besser verstanden werden. Als klinisch relevante Veränderungen wurden neben einer Mikrosatelliteninstabilität bisher IDH-1- und BRAF-Mutationen, FGFR-Alterationen sowie eine HER2-Überexpression identifiziert. Eine Vorstellung der betroffenen Patienten in einem molekularen Tumorboard ist zu empfehlen, um potenzielle zielgerichtete Therapieoptionen sowie ggf. eine Studienteilnahme zu evaluieren.

Aktuelle Therapiestandards Patienten nach einer kurativ intendierten Resektion sollte eine adjuvante Chemotherapie mit Capecitabin über 6 Monate angeboten werden. Die Kombination aus Gemcitabin und Cisplatin bleibt etablierter Standard in der palliativen Erstlinientherapie. Patienten in einem guten Allgemeinzustand kann aufgrund der positiven Ergebnisse der randomisierten ABC-06-Studie eine Zweitlinientherapie empfohlen werden.

Personalisierte Therapie Mehr als 50 % aller Patienten mit malignen biliären Tumoren weisen therapierbare genetische Alterationen auf. Erste prospektive Daten belegen den Nutzen einer zielgerichteten Therapie bei diesen Patienten. Deswegen sollte möglichst allen Patienten, die eine palliative Systemtherapie benötigen, frühzeitig ein molekulares Profiling angeboten werden.

Abstract

Biliary tract cancer (BTC) is a rare disease with a heterogeneous nomenclature. Carcinomas of the intra- and extrahepatic biliary tract as well as gallbladder cancer are oftentimes combined in clinical research and treatment algorithms. However, these different cancer types vary not only in their anatomical features, but also in the underlying molecular alterations.

Surgery remains the only chance of cure. Adjuvant chemotherapy with capecitabine for 6 months should be recommended after curative intended surgery. In the palliative first-line treatment of advanced BTC, the combination chemotherapy gemcitabine and cisplatin remains the only evidence-based standard. For second-line treatment, the combination of 5-FU, folinic acid and oxaliplatin (FOLFOX) is a treatment option based on preliminary data from a randomized phase 3 study. Potential targeted therapies showing efficacy in prospective clinical studies are, for example, IDH-, BRAF-/MEK- and FGFR-inhibitors as well as immunotherapy. Therefore, in the era of personalized medicine, molecular testing should be offered to all patients with advanced disease and indication for systemic treatment.

Schlüsselwörter

maligne biliäre Tumoren - Gallenblasenkarzinom - Cholangiokarzinom - personalisierte Therapie - Chemotherapie

Key words

biliary tract cancer - gallbladder cancer - cholangiocarcinoma - personalized therapy - chemotherapy

Publication History

Article published online:
01 April 2020

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