Hum Pathol 1985 Jul;16(7):719-26

Hepatobiliary carcinoma associated with primary sclerosing cholangitis and
chronic ulcerative colitis

Wee A, Ludwig J, Coffey RJ Jr, LaRusso NF, Wiesner RH

Abstract
Hepatobiliary carcinomas were found in eight patients with chronic ulcerative
colitis (CUC) and primary sclerosing cholangitis (large-duct PSC; five cases) or
"pericholangitis" (small-duct PSC; three cases). The tumors were extrahepatic in
five cases and intrahepatic in two; in one case the neoplasm affected both liver
and gallbladder. The tumors in seven patients were glandular and, sometimes,
cystic and papillary; in the remaining patient a combined hepatocellular
carcinoma and cholangiocarcinoma was found. The latter tumor seemed to arise
from regenerative nodules in secondary biliary cirrhosis complicating PSC. The
presence of carcinoma in situ in areas of fibrous cholangitis, the multicentric
origin of the tumor, the presence of tumor-free large-duct PSC or small-duct PSC
(pericholangitis) at a distance from the carcinomatous areas, and the
documentation, in some cases, of long-standing inflammatory hepatobiliary
disease prior to the discovery of the tumors would seem to confirm the clinical
impression that carcinomas may develop in pre-existing PSC. The appearance of
hepatobiliary carcinomas in patients with classic PSC and in patients with
pericholangitis supports previous evidence indicating that cholangiographically
diagnosed large-duct PSC and histologically diagnosed small-duct PSC
(pericholangitis) are manifestations of a shared condition that could be named
PSC syndrome. The findings of the present study indicate that the PSC syndrome
predisposes patients for the development of bile duct carcinoma. Most patients
with CUC and bile duct carcinoma seem to have PSC prior to the development of
the hepatobiliary tumor