1: Hepatology 1996 May;23(5):1105-11

Biliary malignancies in primary sclerosing cholangitis: timing for live
transplantation

Nashan B, Schlitt HJ, Tusch G, Oldhafer KJ, Ringe B, Wagner S, Pichlmayr R
Klinik fur Abdominal- und Transplantationschirurgie, Medizinische Hochschule
Hannover, Germany.
Abstract
Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease
associated in 10% to 36% of those 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 has not yet been achieved, because
there are no differences in the age of patients or clinical course, particularly
with regard to the time between diagnosis of PSC and detection of carcinomas. To
assess optimal timing for transplantation in patients with PSC, we applied the
Mayo survival model to 48 patients receiving transplants for that disease in our
center between 1972 and 1994. Of these patients, 10 had a biliary malignancy,
which was incidental in 9. According to the Mayo model, low-, moderate-, and
high-risk groups of patients could be formed. The actuarial patient survivals at
1 and 7 years were 100% and 100% (low risk), 68.6% and 68.6% (moderate risk),
and 54.6% and 46.8% (high risk), respectively. Patients with a biliary
malignancy had a 30% survival at 1 year; none survived 6 years. Local recurrence
of the tumor was found in 3 patients, 2 of them with low tumor stages at the
time of transplantation. Analysis of the Mayo Model risk scores demonstrated a
marked increase in the incidence of biliary malignancies at a score above 4.4.
All patients with tumors were found to have a score above 4. Moreover, the
prevalence rate rose from 14.3% in the low-risk group to 33.3% in the
moderate-risk group. There was no difference in the clinical courses at 6 to 12
months prior to transplantation; in particular, the bilirubin levels (PSC alone,
250 +/- 230 mumol/L; PSC with carcinoma, 288 +/- 182 mumol/L) did not differ
significantly (P > .05) between both patient groups. Because the outcome after
transplantation is poor even in patients with low-grade malignancies, early
timing of transplantation in patients with PSC is suggested to prevent formation
of biliary malignancies. Therefore, regular scoring of patients with the Mayo
Model risk score is suggested, and transplantation should be taken into
consideration at scores above 4