Cholangiocarcinoma--current treatment options

Abstract

The treatment for cholangiocarcinoma (CCA) remains a challenge because of the aggressive nature of the disease and the absence of effective treatments besides surgical resection (HR) and liver transplantation (LT). In intrahepatic CCA, HR remains the treatment of choice whereas with concomitant liver disease such as cirrhosis or primary sclerosing cholangitis (PSC), LT is the only option. Hilar CCA or Klatskin tumours have in recent decades been managed with a more aggressive surgical approach to achieve R0 resection. This approach usually involves preoperative portal embolisation, followed by liver resection – sometimes extensive and even with portal vein resection. The recent protocols that combine preoperative neoadjuvant chemoirridation and LT show promising results that need to be confirmed. The development of diagnostic modalities (tumour markers, cytology and radiology) are of the utmost importance to identify these patients at an early stage to preserve radical surgery possible. Cholangiocarcinoma (CCA) is a malignant disease of the epithelial cells in the intra- and extrahepatic bile ducts. While still a rare malignant disease, CCA is the second most common primary malignancy of the liver. The incidence is increasing; especially the incidence of intrahepatic CCA (1). The treatment of CCA is challenging as it is usually difficult to diagnose when radical surgical treatment, resection (HR) or liver transplantation (LT) is possible. The lack of effective medical treatment makes a radical surgical resection or hepatectomy the only therapeutic option. Most of the CCAs are unresectable at presentation and the prognosis for these patients is dismal.