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Fibroinflammatory tumor of the liver causing biliary cirrhosis in an infant
E M Alonso1, T M Zdunek, M R Brown
1Department of Pediatrics, University of Chicago Pritzker School of Medicine, Illinois.
Insights
A rare pediatric liver tumor caused biliary obstruction in a 21-month-old. Liver transplantation successfully treated the fibroinflammatory mass, with no recurrence after 18 months.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Surgical Oncology
Background:
- Fibroinflammatory tumors are rare hepatic neoplasms.
- Biliary obstruction in children can lead to severe complications.
Observation:
- A 21-month-old presented with fever, diarrhea, and jaundice due to a liver mass.
- Imaging revealed a porta hepatis tumor causing common hepatic duct destruction.
Findings:
- Histology confirmed a fibroinflammatory tumor with dense fibrous stroma and inflammatory cell infiltration.
- The tumor caused chronic biliary obstruction, leading to biliary cirrhosis within 4 months.
Implications:
- Early diagnosis and surgical intervention are crucial for pediatric liver tumors.
- Orthotopic liver transplantation offers a viable curative option for unresectable or complex cases.
Abstract:
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.