Treatment Options

There are two objectives in the therapy of hilar cholangiocarcinoma: complete tumor excision with negative margins and subsequent restoration of biliary-enteric continuity. Multiple studies from several centers around the world have shown that complete resection is associated with five-year survival rates of approximately 25% to 40%, clearly better than can be achieved with nonoperative therapies. Clearly, patients treated nonoperatively typically have more advanced disease, and no comparative trials have been performed. Nevertheless, given the relatively poor response rates with chemotherapy and chemoradiation therapy, resection has emerged as the most effective treatment. Orthotopic liver transplantation has been attempted for unresectable hilar tumors. Klempnauer et al. reported four long-term survivors out of 32 patients submitted to transplantation for hilar cholangiocarcinoma (62). The same group also reported a 17.1% five-year survival for their overall transplant group (63). Comparable results

TABLE 2 Criteria of Unresectability Patient factors

Medically unfit or otherwise unable to tolerate a major operation Hepatic cirrhosis Local tumor-related factors Tumor extension to secondary biliary radicles bilaterally Encasement or occlusion of the main portal vein proximal to its bifurcation Atrophy of one hepatic lobe with contralateral portal vein branch encasement or occlusion Atrophy of one hepatic lobe with contralateral tumor extension to secondary biliary radicles Unilateral tumor extension to secondary biliary radicles with contralateral portal vein branch encasement or occlusion Metastatic disease Histologically proven metastases to N2 lymph nodes Lung, liver, or peritoneal metastases

Source: From Ref. 57.

TABLE 3 Summary of Selected Studies Showing the Relationship Between the Rate of Partial Hepatectomy and Proportion of Negative Histologic Margins Achieved

Complete gross Partial hepatectomy Negative margin

Tsao (2000) 25 16 28

Cameron (1990) 39 20 15

Gerhards (2000) 112 29 14

Hadjis (1990) 27 60 56

Jarnagin (2001) 80 78 78

Klempnauer (1997) 147 79 79

Neuhaus (2000) 95 85 61

Nimura (1990) 55 98 83

were reported by Iwatsuki et al. (64). The results of transplantation have previously not been sufficiently adequate to justify its use, and most centers now do not perform liver transplantation for cholangiocarcinoma. More recently, data from the Mayo Clinic have emerged suggesting good results with transplantation in highly selected patients with low volume unresectable disease and combined with an intensive pretransplant treatment regimen (65,66). Although the data are compelling, this approach is applicable to a very small fraction of patients.

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