Progress im Management des cholangiozellulären Karzinoms

Liver cancer, especially cholangiocarcinoma (CC), is a significant cause of cancer deaths in Austria. CC is divided into gallbladder and bile duct carcinoma with the latter being further subdivided into intrahepatic (iCC) and extrahepatic (eCC) types. Risk factors include hepatitis infection, liver cirrhosis, bile duct inflammation, and lifestyle factors such as diabetes, fatty liver, as well as nicotine and alcohol consumption. Symptoms, often non-specific and only appearing in advanced stages, include weight loss, weakness, malaise, and abdominal pain. Early signs of eCC include cholestasis, jaundice, dark urine, pruritus, and pale stools. Tumor markers such as Carbohydrate-Antigen 19-9 (CA 19‑9), carcinoembryonic antigen (CEA) and alpha-fetoprotein (AFP) are used for diagnosis, although CA 19‑9 and CEA may be elevated in CC. Imaging techniques such as computed tomography, magnetic resonance imaging, endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography (ERCP) play a central role in diagnosis and staging. Histopathologically, CCs are classified as adenocarcinomas, subdivided into “small duct” and “large duct” types, whereby immunohistochemistry is used for differentiation. Systematic identification of treatable mutations is essential for the choice of therapy. Treatment depends on the stage of the tumor. For localized tumors, surgery is the first choice, supported by preoperative chemotherapy in certain cases. Advanced tumors require systemic therapies such as gemcitabine and cisplatin or molecular targeted therapies, depending on specific mutations. Immunotherapy is also gaining in importance. Treatment of advanced cancer with high microsatellite instability often includes pembrolizumab. Advances in molecular biology-based therapies and immunotherapy promise improved treatment options in the future for patients with CC.