Survival rates

Hilar cholangiocarcinoma, also called perihilar cholangiocarcinoma or Klatskin tumor, is an aggressive cancer that often grows silently until it blocks the bile ducts. Because many people are diagnosed after the disease has already spread, the overall survival rate remains low.

After surgery

Surgery that completely takes out the cancer, often called R0 resection, is the only treatment that offers a chance for long-term survival. When cancer cells are found at the edges of the removed tissue, called an R1 or R2 resection, the survival rate is lower. Survival also is lower when the cancer has spread to lymph nodes or blood vessels.

If the cancer cannot be taken out

If surgery is not possible, treatment focuses on easing symptoms and slowing the cancer's growth. These therapies are not expected to cure the cancer, but they can help improve quality of life and extend survival.

Treatments often include chemotherapy or immunotherapy. Chemotherapy is usually the first treatment offered, and immunotherapy may be considered depending on the tumor's genetic features. Most people in this situation live between six and 12 months, even with treatment.

After liver transplantation

Some people with small, localized tumors that cannot be removed may be offered liver transplantation at specialized centers. In these people, cancer has not spread to lymph nodes or distant organs and they have shown a good response to chemoradiation before transplantation. For some people, outcomes after transplant can be better than those after surgery, such as for people who also have primary sclerosing cholangitis.

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