Background: Differentiating primary sclerosing cholangitis (PSC) as a result of cholangiocarcinoma (CCC) can be a diagnostic challenge with major therapeutic implications. In case of advanced or symptomatic PSC, liver transplantation (OLTx) can be life saving with excellent long-term outcome.
However, the outcome of CCC diagnosed prior or during OLTx is disastrous.
Objective: The purpose of this study was to assess the value and timing of OLTx in the treatment of PSC with incidental cholangiocarcinoma.
Material/Methods: 686 patients underwent liver transplantation in Department
of General, Transplant and Liver Surgery, Medical University of Warsaw until
31.12.2008. Malignant neoplasm was an indication in 85 cases. A retrospective study was carried out, including all cases of OLTx for PSC performed between June 1998 and December 2008. In the group of all 59 cases of PSC 4 pts with cholangiocarcinoma were found in postoperative histopathology. 2 female and 2 male, 32, 40, 36 and 29 years of age. Those were respectively: 1cm tumour in the hilus of the liver, 3.5 cm tumour in the right hepatic duct, 1.5 cm tumour in the IIIrd segment of the liver and in one case 5 tumours disseminated in both lobes with maximum 2cm in diameter each. Ca19.9 serum concentrations before OLTx (IU/ml) were 157, 1200, n/d and 13, when in the whole PSC group 26±21 was observed. All patients with CCC received chemotherapy: gemcitabine/FAM (5-Fu + Doxorubicin + Mitomycin). In immunosuppressive therapy the predominated regimen were steroids and tacrolimus converted to rapamycine in cases of CCC.
Results: Patients with cholangiocarcinoma died after 4, 15 and 19 months after OLTx due to dissemination of neoplasmatic process; last OLTx was made in December 2008 and until now the patient is without symptoms of recur-rence. In comparison, in group with PSC 8 patients died (14.5%), median survival 33±28m (follow-up range 1-120 m).
Conclusions: There is evidence that early transplantation, before end stage
liver disease or until cholangiocarcinoma has developed, improves the survival of patients with PSC. Ca19.9 serum concentrations may be one of the prognostic factors. Because it is the only procedure of proven benefit, patients with PSC should be considered for liver transplantation early in the course of the disease.

Keywords: Liver Transplantation