Transplantation for Cholangiocarcinoma
Section snippets
Risk factors
Although most cases of CCA arise in patients without an identifiable risk factor (ie, de novo CCA), several risk factors for the development of CCA have been identified, with chronic biliary inflammation being a common feature shared by many of them. Primary sclerosing cholangitis (PSC) is the most common identifiable risk factor in Western countries. The incidence of CCA in patients with PSC is 0.6% to 1.5% per year.6, 7 The lifetime risk of developing CCA in patients with PSC is between 7%
Pathophysiology and classification
Greater than 90% of CCAs are adenocarcinomas.18 Typically CCA is a well-differentiated to moderately differentiated adenocarcinoma with a prominent, dense, desmoplastic stroma (Fig. 1). This stroma leads to annular thickening of the bile duct caused by the infiltration and fibrosis of the periductal tissues. The large amount of fibrous stroma greatly increases the difficulty in confirming CCA by cytology and biopsy. Uncommon histologic variants include papillary adenocarcinoma, squamous cell,
Chemotherapy
Several chemotherapeutic regimens have been used in the treatment of CCA. However, the currently available chemotherapeutic or radiation therapy regimens only affect a modest improvement in patient survival. Most studies are difficult to interpret because of the small numbers of patients in each series and the inclusion of other neoplasms, such as gallbladder, pancreas, or hepatocellular carcinoma, which likely have a different response. Several regimens have used infusional 5-fluorouracil
Surgical resection
Surgical resection provides the only potential curative therapy for CCA. Unfortunately, CCA usually presents late in the disease course, and most patients have unresectable disease at presentation. Criteria for unresectability include distant lymph node or liver metastases; predicted inadequate function in the liver remnant; and bilobar involvement, including bilateral hepatic duct involvement of the secondary biliary radicals or atrophy of one lobe of the liver with encasement of the
Liver transplantation
Initial experiences with orthotropic liver transplantation (OLT) for unresectable CCA were disappointing because of the frequent recurrence of CCA. The 5-year survival rate was only 5% to 15% in these early studies.51, 52 However, there were some long-term survivors among patients with negative surgical resection margins and negative regional lymph nodes.53 This finding prompted the development of a protocol at the University of Nebraska and Mayo Clinic for neoadjuvant chemoradiation followed
Summary
CCA is a rare malignancy that arises from malignant transformation of the cells that line the biliary tree. The incidence of CCA is increasing globally. Primary sclerosing cholangitis and other conditions with chronic inflammation of the biliary tree are risk factors for the development of CCA. Hilar CCA usually presents with obstructive jaundice. Diagnostic techniques have significantly improved in the past 2 decades, enabling more accurate and less-invasive evaluation. Surgical resection and
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