SEGMENTAL PERICHOLANGIAL FIBROSIS - A PECULIAR BENIGN FIBROSING DISEASE AT THE HEPATIC HILUM

The authors report on a 9-year-old child who underwent surgery to remo ve a tumor of the hepatic hilum with preoperative radiographic studies suggestive of malignancy, but whose surgical specimens showed a pecul iar fibrosing disease. The lesion was localized to the bifurcation of the hepatic duct, where the bile duct wall and the surrounding tissue was markedly fibrotic. No malignant cells or epithelial destruction we re seen, The patient's postoperative course was uneventful, and he is without any sign of recurrence 2 years after surgery. Because the hist ological features of this case do not correspond to any established di sease, including primary sclerosing cholangitis, the authors believe i t represents a new entity, segmental pericholangial fibrosis. Local re section resulted in a good outcome. A review of the literature disclos ed a few similar cases with a benign clinical course. Copyright (C) 19 97 by W.B. Saunders Company.