Idiopathic Retroperitoneal Fibrosis and Sclerosing Cholangitis Associated with a Reticulum Cell Sarcoma: Report of a case

The case of a 16-year-old girl is described who developed sclerosing cholangitis, involving the common bile duct and hepatic hilum, associated with but physically separate from a rapidly enlarging paracholedochal primitive reticulum cell sarcoma. Serial cholangiograms performed via an indwelling gallbladder catheter and a hepatic duct catheter afforded the unique opportunity to observe the parallel development of these lesions. An additional finding at autopsy was the presence of typical periureteral retroperitoneal fibrosis. The findings of this case are consistent with previous reports of the concomitance of sclerosing cholangitis and retroperitoneal fibrosis. The association of these sclerosing processes with a malignant tumor has not been previously described.