Cholangiocarcinoma is a cancer of the bile ducts, which drain bile from the liver into the small intestine. It is a relatively rare cancer, with an annual incidence. If the tumor cannot be surgically removed, patients are often treated with palliative chemotherapy with or without radiotherapy. Chemotherapy has been shown in a randomized controlled trial to improve quality of life and extend survival in patients with inoperable cholangiocarcinoma. There is no single chemotherapy regimen which is universally used, and enrollment in clinical trials is often recommended when possible. Chemotherapy agents used to treat cholangiocarcinoma include 5-fluorouracil with leucovorin, gemcitabine as a single agent, or gemcitabine plus cisplatin, irinotecan, or capecitabine. Combined chemoradiation, although often done, is not proven and NCCN recommends it on a clinical trial. PDQ says, “For patients with locally unresectable tumors, preoperative radiation therapy with various chemotherapeutic agents and/or radiosensitizers is under clinical evaluation.” A recent guideline in Gut, referenced below states : “The role of chemoradiation (chemotherapy combined with local radiation) remains to be established in randomised clinical trials as local and systemic toxicity is also concomitantly increased.” As per paln language, it is evident that theh consensus of experts is that clinical nvestigtion is required for this approach. It is , therefore, investigational. NCCN, however, lists chemoradiation as a standard recommendation and it is a later guideline.

http://www.nccn.org/professionals/physician_gls/PDF/hepatobiliary.pdf

http://www.cancer.gov/cancertopics/pdq/treatment/pancreatic/HealthProfessional/page7/print

S A Khan et al Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document Gut 2002;51

Chapman R, Fevery J, Kalloo A, Nagorney DM, Boberg KM, Shneider B, Gores GJ, American Association for the Study of Liver Diseases. Diagnosis and management of primary sclerosing cholangitis. Hepatology 2010 Feb;51(2):660-78. http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/PSC_2-2010.pdf

Ali Shorbagi, Yusuf Bayraktar Primary sclerosing cholangitis – What is the difference between east and west? World J Gastroenterol  2008 July 7; 14(25): 3974-3981

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