Hepatoblastoma and Abernethy Malformation Type I: Case Report
Catalina Correa1, Juan P Luengas, Scott C Howard
1*Instituto Nacional de Cancerología, Bogota, Colombia †School of Health Studies, University of Memphis, Memphis, TN.
Journal of Pediatric Hematology/Oncology
|July 29, 2016
Summary
Congenital portosystemic shunts, like Abernethy malformation type I, are linked to liver tumors. These shunts may increase the risk of liver failure after surgery for hepatoblastoma.
Area of Science:
- Hepatobiliary surgery
- Pediatric oncology
- Vascular malformations
Background:
- Congenital portosystemic shunts, specifically Abernethy malformation type I, are rare vascular anomalies.
- These malformations can be associated with the development of hepatic tumors, posing unique clinical challenges.
Observation:
- A 2-year-old boy with pneumonia was incidentally found to have a large hepatic mass (PRETEXT III).
- He also had a congenital absence of the portal vein with drainage to the inferior vena cava (Abernethy malformation type I).
Findings:
- The patient was diagnosed with hepatoblastoma and received neoadjuvant chemotherapy and chemoembolization, downstaging the tumor to POSTEXT III.
- Despite surgical resection (right extended hepatic lobectomy), the patient experienced fatal hepatic failure postoperatively.
- The Abernethy malformation type I was identified as a potential contributing factor to the poor surgical outcome.
Implications:
- This case highlights the association between Abernethy malformation type I and hepatoblastoma.
- Abnormal hepatic vasculature in these patients may increase the risk of hepatic failure following liver resection.
- Thorough vascular assessment is crucial in patients with congenital portosystemic shunts and liver tumors.
- Liver transplantation may be a viable option for select patients with these complex conditions.
