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Department of Pathology Case of the Month May 2002 38-year old man with jaundice, intense pruritus and dark urine Allison Miller, W. W. L. Chang M.D., Ph.D
Microscopic Description The microscopic examination of the liver at autopsy showed rare areas of degenerating interlobular bile ducts, which were associated with a dense “onion-skin” periductal fibrosis (figure 4). There were also nodular fibrotic areas corresponding to the site of previous interlobular or segmental ducts (figure 5) where the bile ducts had been obliterated by the progression of sclerosis and had disappeared. Progressive destruction and sclerosis of bile ducts had caused rupture of the remaining bile ducts due to increased pressure and spillage of bile into the surrounding areas known as bile lakes (figure 6), which led to bile infarcts (figure 7). The latter were characterized by cystic changes with varying numbers of foamy, bile-laden macrophages and cell debris. Secondary abscess formation was also present. The liver in general exhibited diffuse cirrhosis with marked bile ductular proliferation and prominent cholestasis, representing secondary biliary cirrhosis (figure 8). The trichrome stain demonstrated the cirrhosis (figure 9). Of importance in this case was the presence of tumor as demonstrated in figures 9 and 10. Neoplastic glandular structures of varied sizes containing mucoid material and/or bile pigment in varying amounts were present in and around the site of intrahepatic bile ducts and invaded into the adjacent hepatic lobules, consistent with the primary origin in bile ducts. Metastatic tumor was demonstrated in the lymph nodes in the porta hepatis and mesentery as well as visceral and parietal peritoneum. The mucosa of the intestines was autolyzed, but evidence of Crohn’s disease was not present at autopsy.
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