Lecture: cholangiocarcinoma

Today’s lecture focused on the topic of cholangiocarcinoma (CCA).  It is a rare tumor, and nearly 70% of them occur sporadically. However, there are several known risk factors, with the most common one being primary sclerosing cholangitis (PSC).  Many cases of CCA are diagnosed within the first two years of a PSC diagnosis.

Cholangiocarcinoma is generally divided into three categories based on location.  These include intrahepatic, hilar (ie. Klatzkin tumor) and distal.  Regardless of location, the five-year prognosis is generally poor, <50%.  Intrahepatic lesions come in several phenotypes, from a papillary (intraductal) to a parenchymal mass lesion.

Diagnosis is difficult.  Imaging is at best 70% sensitive, and ductal infiltrating lesions are particularly hard to pick up.  Biomarkers also have poor sensitivity, and a CA 19-9 is best when > 129.  The specificity is worsened by the presence of cholangitis or ductal obstruction.

Treatment options depend on the extent of the tumor.  If caught early, surgical resection or liver transplantation is possible at a center with expertise.  Local ablation therapy can be used and experience there is limited.  Systemic chemotherapy can also be attempted.  Liver transplantation is not being performed at 12 centers across the country, and the protocol is extensive; it includes brachytherapy, chemotherapy and exploratory laparotomy before liver transplant is possible.  There can be no metastatic disease.

The main take home point is that, for CCA, diagnosis and treatment are difficult.  More research is needed!

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