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Clinical Description: Cholangiocarcinoma

Similar to gallbladder cancer, the cancer of the bile ducts, called cholangiocarcinoma, is a rare entity accounting for only 3% of gastrointestinal cancer cases and 2 in 100,000 people in the United States. Choangiocarcinomas usually become symptomatic when they obstruct the biliary drainage system, causing painless jaundice.

Symptoms of Cholangiocarcinoma Cancer

Other symptoms include: pruritus (itching), abdominal pain, fever and weight loss. Unfortunately, symptoms usually arise late in the disease and patients present with advanced cancer at diagnosis.

Risk Factors of Cholangiocarcinoma Cancer

Primary sclerosing cholangitis, Lynch syndrome, infection with certain parasites and having chronic liver disease, are some of the risk factors associated with Cholangiocarcinoma.

How Cholangiocarcinoma Cancer is Diagnosed

The initial evaluation will include a physical exam, laboratory and radiological studies. Laboratory studies will suggest a blockage of the biliary system, having increased levels of alkaline phosphatase and direct bilirubin. Tumor markers such as CEA and CA 19-9 can also be increased and help in the diagnosis, but they are not specific for bile duct cancers. To attain the diagnosis, imaging studies and a biopsy should be performed since benign conditions and other types of malignancy can present similarly.

Evaluation includes abdominal ultrasound, CT scan, magnetic resonance cholangiography (MRCP), or endoscopic retrograde cholangiopancreatography (ERCP). These tests help doctors localize the extent of the disease. Biopsy can be obtained either by ERCP, through the skin (percutaneous) guided through a CT scan or surgically as a last approach.

Treatment Options for Cholangiocarcinoma at Tufts Medical Center

Once the diagnosis is confirmed, surgical resection gives the only chance of cure, but treatment with a combination of chemotherapy and/or radiation therapy can also help control the disease. Surgery would involve removal of part of the bile duct and possibly removal of part of the liver. Chemotherapy agents such as gemcitabine, cisplatin, 5-fluorouracil and erlotinib may be useful. Novel treatment approaches are being used. Palliative measures such as endoscopic stents to relieve biliary obstruction can help with symptom control.

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