is suggested, and transplantation should be taken into Primary sclerosing cholangitis (PSC) is a chronic inconsideration at scores above 4. (HEPATOLOGY 1996; flammatory disease associated in 10% to 36% of those 23:1105-1111.) with hepatobiliary malignancies, which are, in the majority of cases, not known prior to transplantation. Diagnosis of carcinomas in a PSC setting at an early stage Primary sclerosing cholangitis (PSC) is a chronic in-
has not yet been achieved, because there are no differflammatory disease that is characterized by multiple ences in the age of patients or clinical course, particufibrotic strictures of the intra-and extrahepatic biliary larly with regard to the time between diagnosis of PSC tree. It is often associated with chronic inflammatory and detection of carcinomas. To assess optimal timing bowel disease (IBD), i.e., ulcerative colitis and Crohn's for transplantation in patients with PSC, we applied the disease, 1-4 or complicated by the presence of carcinomas Mayo survival model to 48 patients receiving transplants of the biliary system, i.e., intrahepatic cholangiocellufor that disease in our center between 1972 and 1994. Of lar carcinomas (CCC), gallbladder carcinomas (GBC), these patients, 10 had a biliary malignancy, which was and extrahepatic bile duct carcinomas (BDC). [5][6][7][8]13 The incidental in 9. According to the Mayo model, low-, moddisease generally progresses slowly, starting in early erate-, and high-risk groups of patients could be formed.
The actuarial patient survivals at 1 and 7 years were adulthood and sometimes even in infancy. It affects 100% and 100% (low risk), 68.6% and 68.6% (moderate two thirds of men and one third of women and frerisk), and 54.6% and 46.8% (high risk), respectively. Paquently leads to cirrhosis, portal hypertension, and tients with a biliary malignancy had a 30% survival at 1 liver failure. year; none survived 6 years. Local recurrence of the tu-A variety of antiinflammatory and immunosuppresmor was found in 3 patients, 2 of them with low tumor sive treatments were applied to PSC, but they have stages at the time of transplantation. Analysis of the been proven to be ineffective. Large series from differ-Mayo Model risk scores demonstrated a marked inent centers 9-12 demonstrate a high actuarial survival crease in the incidence of biliary malignancies at a score after liver transplantation for PSC. Particularly by above 4.4. All patients with tumors were found to have careful selection and early timing of transplantation,
a score above 4. Moreover, the prevalence rate rose from 14.3% in the low-risk group to 33.3% in the moderate-risk excellent results can be achieved, compared with the group. There was no difference in the clinical courses at estimated survival calculated by specific risk scores 6 to 12 months prior to transplantation; in particular, based on the natural history of PSC. 9,14 In this context, the bilirubin levels (PSC alone, 250 { 230 mmol/L; PSC the observation of a 10% to 36% incidence of biliary with carcinoma, 288 { 182 mmol/L) did not differ significarcinomas in patients with PSC at the time of liver cantly (P ΓΊ .05) between both patient groups. Because transplantation is of interest. 4,7,6,[10][11][12]14 The prognosis the outcome after transplantation is poor even in pafor patients in whom biliary carcinomas develop in the tients with low-grade malignancies, early timing of setting of PSC is poor, with a median survival of only transplantation in patients with PSC is suggested to pre-7 months. 9 Liver transplantation is assumed not to be vent formation of biliary malignancies. Therefore, regueffective for the treatment of this constellation, 10 belar scoring of patients with the Mayo Model risk score cause recurrence of the tumor limits the prognosis of the patients. Thus, the prognosis depends on early diagnosis, which, with the existing diagnostic tools, is Abbreviations: PSC, primary sclerosing cholangitis; IBD, inflammatory bowel disease; CCC, cholangiocellular carcinoma; GBC, gallbladder carcinoma; not possible. [13][14][15] BDC, bile duct carcinoma.
To assess timing in PSC patients, with particular From the 1 Klinik fu Β¨r Abdominal-und Transplantationschirurgie, 2 Institut focus on the poor outcome in patients with incidental fu Β¨r Biometrie, and 3 Klinik fu Β¨r Gastroenterolgie und Hepatologie, Medizinische carcinoma, we retrospectively evaluated our trans-