Cholangiocarcinoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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 of 1–2 cases per 100,000 in the Western world,[1] but rates of cholangiocarcinoma have been rising worldwide over the past several decades.[2] Risk factors for cholangiocarcinoma include primary sclerosing cholangitis (an inflammatory disease of the bile ducts), congenital liver malformations, infection with the parasitic liver flukes Opisthorchis viverrini or Clonorchis sinensis, and exposure to Thorotrast (thorium dioxide), a chemical previously used in medical imaging. The symptoms of cholangiocarcinoma include jaundice, weight loss, and sometimes generalized itching. The disease is diagnosed through a combination of blood tests, imaging, endoscopy, and sometimes surgical exploration.
Surgery is the only potentially curative treatment, but most patients have advanced and inoperable disease at the time of diagnosis. After surgery, adjuvant chemotherapy or radiation therapy may be given to increase the chances of cure. Patients with advanced and inoperable cholangiocarcinoma are generally treated with chemotherapy and palliative care measures. Areas of ongoing medical research in cholangiocarcinoma include the use of newer targeted therapies (such as erlotinib) and the use of photodynamic therapy.
Diagnosis
Cholangiocarcinoma is definitively diagnosed from tissue, i.e. it is proven by biopsy or examination of the tissue excised at surgery. It may be suspected in a patient with obstructive jaundice. Considering it as the working-diagnosis may be challenging in patients with primary sclerosing cholangitis (PSC); such patients are at high risk of developing cholangiocarcinoma, but the symptoms may be difficult to distinguish from those of PSC. Furthermore, in patients with PSC, such diagnostic clues as a visible mass on imaging or biliary ductal dilatation may not be evident.
Treatment
Cholangiocarcinoma is considered curable only by surgical removal. Without surgery, it is a rapidly fatal disease with 5-year survival rates of less than 5%.[3] Often, the operability of the tumor can only be assessed at the time of surgery;[4] therefore, most patients undergo exploratory surgery unless there is a clear-cut indication that the tumor is inoperable.[5]
Adjuvant therapy followed by liver transplantation may have a role in treatment of certain unresectable cases.[6]
References
- ↑ Landis S, Murray T, Bolden S, Wingo P. "Cancer statistics, 1998". CA Cancer J Clin. 48 (1): 6–29. PMID 9449931.
- ↑ Patel T. "Worldwide trends in mortality from biliary tract malignancies". BMC Cancer. 2: 10. PMID 11991810.
- ↑ Farley D, Weaver A, Nagorney D (1995). ""Natural history" of unresected cholangiocarcinoma: patient outcome after noncurative intervention". Mayo Clin Proc. 70 (5): 425–9. PMID 7537346.
- ↑ Su C, Tsay S, Wu C, Shyr Y, King K, Lee C, Lui W, Liu T, P'eng F (1996). "Factors influencing postoperative morbidity, mortality, and survival after resection for hilar cholangiocarcinoma". Ann Surg. 223 (4): 384–94. PMID 8633917.
- ↑
- ↑ Heimbach JK, Gores GJ, Haddock MG, et al, Predictors of disease recurrence following neoadjuvant chemoradiotherapy and liver transplantation for unresectable perihilar cholangiocarcinoma, Transplantation. 2006 Dec 27;82(12):1703-7.