Fibroinflammatory tumor of the liver causing biliary cirrhosis in an infant.

We report the case of a 21-month-old child with a fibroinflammatory tumor of the liver causing biliary obstruction. The patient had fever, diarrhea, and jaundice. Abdominal ultrasonography, computerized tomography, and endoscopic retrograde cholangiopancreatography demonstrated the tumor to be in the porta hepatis. Growth of the mass resulted in destruction of the common hepatic duct, and chronic obstruction led to biliary cirrhosis over 4 months' time. Histologic study of the tumor revealed a fibrous stroma infiltrated by inflammatory cells. The patient was successfully treated with an orthotopic liver transplantation and is doing well 18 months following initial diagnosis without evidence of recurrence.