Cholangiocarcinoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Suveenkrishna Pothuru, M.B,B.S. [2]

Natural History, Complications, and Prognosis

Natural history

The symptoms of cholangiocarcinoma usually develop in the fourth decade of life, and start with symptoms such as abdominal pain, jaundice and fever.

Complications

Common complications of cholangiocarcinoma include:

• Infection
• Liver failure
• Tumor metastasis

Prognosis

• Depending on the extent of the cholangiocarcinoma at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor with 5-year survival of only 10-44%.
• The prognosis may be worse for patients with primary sclerosing cholangitis who develop cholangiocarcinoma.
• The most important factor in prognosis of cholangiocarcinoma is whether or not the tumor is able to be resected.

Extent of the tumor

• Patients with multiple tumors, larger tumors and tumors that have spread to nearby blood vessels or lymph nodes have a poor outcome.

Resectability

Tumors that can be completely removed by surgery (resectable) have a more favorable prognosis than tumors that cannot be removed by surgery (unresectable).

• Distal cholangiocarcinoma: Long-term survival rates range from 15%–25%.
• Intrahepatic cholangiocarcinoma: Survival estimates after surgery ranging from 22%–66%.
• Perihilar cholangiocarcinoma: 5 years survival rates range from 20%–50%.

Surgical margins

• The best prognostic factors are resection of tumor-free surgical margin without lymph node invasion.
• Tumor diameter, histology, and differentiation are poor predictors of good outcome with 5-year survival rates varying from 20 to 60%.
• For extrahepatic cholangiocarcinoma, 5 year survival rate is approximately 30% after resection of tumor-free surgical margins.Majority of patients have recurrence due to following reasons:
  • Disseminated tumors
  • Formation of new tumors in previously oncogenic liver tissue