gms | German Medical Science

Background: Primary Sclerosing Cholangitis(PSC)is a biliary destructive disease with increased risk to develop cholangiocarcinoma.To date,liver transplantation is the only curative therapy for PSC.However,PSC patients which are at high risk should be accurately selected for transplantation.In an attempt to establish prognostic factors for this selection we investigated the value of screening cytology

Material and Methods: This retrospective study investigated a period of 10 years(6/1996 until 07/2005)at our hospital and identified 120 patients afflicted from PSC and satisfying inclusion criteria. In all patients biliary specimens were obtained by ERC-guided brush cytology and bile aspiration.Survival was calculated by using the Kaplan-Meier algorithm.

Results: This study enrolled 120 patients with PSC(27% female,73% male,mean age42±13(range18-78) years) within a period of 7 years.Bile specimen cytology revealed carcinoma in 14.7% (17/120) carcinoma, high grade dysplasia in (27/120), medium grade dysplasia in 22.7% (20/88) and low grade dysplasia in 5.7% (5/88) as well as inflammation or normal findings in 19.3% (17/88). 5 patients with microscopic cholangiocarcinoma(no tumor signs in image modalities)received liver transplantation.Thereof,1 patient died due to tumor recurrence while 4 patients are still alive giving a mean survival of 2.7 ± 0.3 years.In contrast,4 out of 8 patients with cytological cholangiocarcinoma and conservative management diet within 1 year resulting in a mean survival time of 1.2 ± 0.2 years. 10 patients with high grade dysplasia in the biliary specimen received liver transplantation, too. 1 patient died due to postoperative complications 4 weeks after transplantation while 9 patients are still alive resulting in a mean survival of 4.9 ± 0.6 years. 23 further patients with high grade dysplasia without surgical therapy showed a mean survival of 3.0 years and 6 fatal courses, 4 of them due to tumor progression and 2 due to liver failure.

Conclusion: Screening ERC with cytological investigation of bile specimen was able to identify 13 out of 88 patients with cytological cholangiocarcinoma at a time were no other diagnostic modality was able to detect the tumor.Liver transplantation of patients with cytological cholangiocarcinoma was highly successful since 5 out of 6 patients turned out to be long term survivors. The approach of screening ERC and cytological investigation is promising and could be a life-saving monitoring for PSC patients.