Hepatoblastoma diagnosed in infancy occurring in single-ventricle patients: a case series

Area of Science:

• Pediatric oncology
• Congenital heart disease
• Hepatobiliary medicine

Background:

• Hepatoblastoma is the most frequent primary liver tumor in pediatric populations.
• There is a lack of reported cases of hepatoblastoma within the single-ventricle congenital heart disease (SV-CHD) demographic.
• Single-ventricle physiology presents unique challenges in pediatric care.

Purpose of the Study:

• To report the first documented cases of hepatoblastoma in patients with single-ventricle congenital heart disease.
• To highlight the occurrence of hepatoblastoma in a population previously considered unaffected.
• To raise awareness among clinicians managing SV-CHD patients about this potential comorbidity.

Main Methods:

• Retrospective case series.
• Review of medical records for three pediatric patients diagnosed with hepatoblastoma.
• Analysis of patient demographics, clinical presentation, and diagnostic timelines, specifically noting the stage relative to Fontan completion.

Main Results:

• Three cases of hepatoblastoma were identified in patients with single-ventricle congenital heart disease.
• The mean age at diagnosis was 19.7 months (±4 months).
• Hepatoblastoma diagnosis occurred before Fontan procedure completion in all three patients.

Conclusions:

• This study presents the only documented instances of hepatoblastoma in patients with single-ventricle congenital heart disease.
• The findings suggest a potential, albeit rare, association between SV-CHD and hepatoblastoma.
• Further research is warranted to explore potential underlying mechanisms and clinical implications for this specific patient group.