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Hepatoblastoma and prune belly syndrome: a potential association
Brian Becknell1, Priya Pais, Grace Onimoe
1Department of Pediatrics, Ohio State University College of Medicine, Columbus, OH 43205, USA. Michael.Becknell@nationwidechildrens.org
Insights
Prune belly syndrome (PBS), a congenital condition, is now potentially associated with hepatoblastoma (HBL), a rare childhood liver cancer. This study identifies four pediatric cases, suggesting a novel link between these conditions.
Area of Science:
- Pediatric Oncology
- Congenital Anomalies
- Hepatobiliary Malignancies
Background:
- Prune belly syndrome (PBS) is a rare congenital disorder affecting the urinary tract and abdominal muscles.
- Malignancies in PBS patients have historically been limited to germ cell tumors.
- Hepatoblastoma (HBL) is the most common primary liver cancer in children.
Observation:
- This report details four pediatric patients diagnosed with both PBS and HBL.
- All identified patients were born after 2002.
- The patients lacked known genetic, natal, or environmental risk factors for HBL.
Findings:
- The co-occurrence of PBS and HBL in these pediatric cases is a novel observation.
- This finding suggests a potential, previously unrecognized association between PBS and hepatoblastoma.
- The absence of typical HBL risk factors in these patients warrants further investigation.
Implications:
- This potential association may necessitate revised surveillance protocols for children with PBS.
- Further research is crucial to elucidate the underlying mechanisms connecting PBS and HBL.
- Understanding this link could improve early detection and management strategies for affected children.
Abstract:
Prune belly syndrome (PBS) is a congenital anomaly characterized by the clinical triad of lax abdominal musculature, bilateral cryptorchidism, and abnormalities of the kidney and urinary tract. Previous reports of malignancy in patients with PBS have been limited to germ cell tumors. Hepatoblastoma (HBL) is the most common hepatic malignancy of childhood, affecting approximately 100 children each year in the USA. We describe a set of 4 pediatric patients with PBS and HBL. All individuals were born after 2002. These subjects lacked genetic, natal, or environmental factors known to confer risk of HBL. The occurrence of PBS and HBL in these patients constitutes a novel potential association.
