Cholangiocarcinoma
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| ICD-10 | C22.1 |
|---|---|
| ICD-9 | 155.1, 156.1 |
| ICD-O: | 8160/3 |
Contents |
[edit] Introduction
Cholangiocarcinoma is an adenocarcinoma of the biliary duct system. It is a relatively rare cancer with an incidence of 1-2:100,000 in the Western world. Its risk factors may include environmental exposures such as polyvinyl chloride or Thorotrast (thorium dioxide); however, this is controversial. It is also associated with the parasite opisthorchis viverrini and clonorchiasis - liver fluke. Other risk factors include hepatolithiasis, congenital liver disorders, thorotrast and ulcerative colitis. A third of patients with primary sclerosing cholangitis will develop cholangiocarcinoma, so careful clinical, laboratory (liver function tests and cancer markers such as CA-19-9), and radiologic follow-up of these patients is warranted. It is NOT associated with hepatitis B or liver cirrhosis. Mutations at the INK4a/ARF locus have been detected in cholangiocarcinomas.
[edit] Treatment
Cholangiocarcinoma is considered curable only by surgical resection, and very often the disease is discovered too late for successful surgery. Chemotherapy has traditionally been seen as largely ineffective, but the chemotherapy agent gemcitabine (Gemzar) is often prescribed. Recently, there has been some success with the GFLIP protocol (Gemzar, 5-FU, leucovorin, irinotecan, and cisplatin or oxaliplatin), a protocol first developed and shown to be useful for pancreatic cancer.
[edit] Prognosis
Less than 10% of patients are considered suitable for surgical resection. Even following resection with negative margins, five year survival rates are are less than 50%.
[edit] See also
[edit] External links
- Cholangiocarcinoma Support Group Forums
- cholangiocarcinoma.org :: a resource for patients, friends, caregivers and loved ones.
[edit] References
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