Klatskin Tumor

Klatskin tumors are a type of cholangiocarcinoma. Cholangiocarcinoma is cancer of the bile duct system in the liver. Dr. Klatskin first described this tumor in 1965. It is located at the liver hilum. The hilum is where the left and right bile ducts join and leave the liver. These two ducts join to make one duct that drains into the bowel.  

What causes a Klatskin tumor?

It is not known what causes a Klatskin tumor. There are risk factors that can cause bile duct system inflammation that could be related to Klatskin tumor development. These risk factors include: primary sclerosing cholangitis, parasitic infections, ulcerative colitis, and choledochal cysts.

What are the symptoms of a Klatskin tumor?

Symptoms are often caused by the tumor blocking drainage from the bile duct. These include:

• Jaundice, which is the buildup of bilirubin that leads to yellowing of the skin and eyes. 
• Clay-colored stools are caused by the lack of bile in the bowel. Bile normally darkens the stool. 
• Bilirubinuria which is dark-colored urine. 
• Pruritus (itchy skin). 
• Weight loss. 
• Abdominal pain. 

Jaundice is the most common symptom of cholangiocarcinomas and is often the presenting symptom. A mass or tumor can be identified on a CT scan or MRI. Once a mass or tumor is identified, a biopsy is needed to confirm the diagnosis. Endoscopic retrograde cholangiopancreatography (ERCP) is a procedure that is used to take a biopsy of a bile duct mass, and it can allow placement of a stent to open the duct and relieve jaundice.

How is a Klatskin tumor treated?

Treatment options include surgery, liver-directed therapy, radiation with or without chemotherapy, chemotherapy, and targeted therapy. Klatskin tumors can involve the liver and one or both bile ducts. If the tumor only involves one side of the liver and/or one side of the bile duct, it may be possible to surgically remove it. If surgery is not possible, radiation therapy can be used with or without low-dose chemotherapy. Low-dose chemotherapy can make the tissue more sensitive to the radiation. Liver-directed therapy such as chemoembolization or radioembolization can be administered also if confined to one lobe of the liver but is not able to be surgically removed.  Chemotherapy can be used with radiation or as the main treatment when the tumor is not able to be removed. Chemotherapy medications that are used include: fluorouracil, gemcitabine, cisplatin, capecitabine, and oxaliplatin. Targeted therapies that can be used based on molecular testing and they include:

• NTRK gene fusion-positive tumors: entrectinib and larotrectinib.
• MSI high or dMMR tumors: pembrolizumab and dostarlimab-gxly.
• BRAF-V600 mutated tumors: dabrafenib with trametinib.
• FGFR 2 fusions or rearrangements: pemigatinib and infigratinib.
• IDHI mutations: ivosidenib.

Patients who have not previously been treated with immunotherapy (or checkpoint inhibitors) may be prescribed nivolumab, lenvatinib, and pembrolizumab.

Treatment is specific to your case. Make sure to talk to your provider about any questions or concerns you have.