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Published on: May 7, 2015
Hepatoblastoma in Children With Congenital Portosystemic Shunts
Timothy B Lautz1, Sonam A Shah, Riccardo A Superina
1Department of Surgery, Ann & Robert H Lurie Children's Hospital of Chicago, Feinberg School of Medicine of Northwestern University, Chicago, IL.
Insights
Hepatoblastoma, a rare liver cancer, can occur with congenital portosystemic shunts (PSSs). Early diagnosis and treatment are crucial for affected children with Abernethy malformations.
Area of Science:
- Pediatric oncology
- Hepatology
- Vascular malformations
Background:
- Congenital portosystemic shunts (PSSs), also known as Abernethy malformations, are rare vascular anomalies.
- While benign hepatic masses are common in Abernethy malformations, the potential for malignant tumors exists.
- Early recognition and management are critical for improving outcomes in affected children.
Observation:
- Two pediatric cases presented with hepatoblastoma concurrently with congenital portosystemic shunts (PSSs).
- Both patients underwent surgical ligation of their PSSs.
- Treatment strategies varied, including concurrent tumor resection or neoadjuvant chemotherapy for initially unresectable tumors.
Findings:
- Hepatoblastoma, a malignant liver tumor, can arise in the context of Abernethy malformations.
- Successful management involved a combination of surgical intervention and chemotherapy.
- This highlights the importance of considering malignancy in the differential diagnosis.
Implications:
- Congenital portosystemic shunts (PSSs) should prompt consideration of associated malignant hepatic masses.
- Multidisciplinary management is essential for treating hepatoblastoma in patients with Abernethy malformations.
- Further research is needed to understand the oncogenesis of hepatoblastoma in PSS patients.
Abstract:
Two children developed hepatoblastoma concurrent with congenital portosystemic shunts (PSSs) (Abernethy malformations). Both underwent operative ligation of their PSSs. One received concurrent tumor resection, whereas the other was deemed initially unresectable and underwent biopsy followed by neoadjuvant chemotherapy. Although benign hepatic masses, such as focal nodular hyperplasia and nodular regenerative hyperplasia, are common in patients with Abernethy malformations, malignant tumors have also been documented and should always be considered in the differential diagnosis of a patient with a congenital PSS and a hepatic mass.
