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A Case of Congenital Hepatoblastoma Coexisting with Pulmonary Hypertension
Zeynep Canan Özdemir1, Adviye Çakıl Sağlık2, Yeter Düzenli Kar1
1Division of Pediatric Hematology/Oncology, Department of Pediatrics, Faculty of Medicine, Eskişehir Osmangazi University, Eskişehir, Turkey.
Insights
Congenital hepatoblastoma (HBL) in infants can cause severe pulmonary hypertension (PHT), negatively impacting prognosis. This rare embryonic liver tumor requires early detection and management strategies for improved outcomes.
Area of Science:
- Pediatric Oncology
- Hepatobiliary Medicine
- Neonatal Care
Background:
- Hepatoblastoma (HBL) is the most common malignant liver tumor in children.
- Congenital HBLs are diagnosed antenatally or within the first three months of life.
Observation:
- A five-day-old infant presented with a large hepatic mass detected during gestation, leading to respiratory distress at birth.
- Echocardiography revealed pulmonary hypertension (PHT) unresponsive to medical therapy.
- Histological analysis showed a mixed epithelial-mesenchymal HBL with a predominant fetal epithelial component.
Findings:
- The infant received chemotherapy on postnatal day 15.
- The patient succumbed to respiratory failure on postnatal day 23.
- This case highlights the potential for HBL to cause severe PHT in neonates.
Implications:
- Hepatoblastoma is an embryonal tumor that can manifest early in development.
- The association between HBL and PHT suggests a potential mechanism impacting prognosis.
- Further research is needed to understand and mitigate the effects of PHT in congenital HBL.
Abstract:
Hepatoblastoma (HBL) is the most frequently detected malignant tumor of the liver in childhood. HBLs detected antenatally or up to 3 months after birth are considered congenital HBLs. We report a five-day-old female infant in whom a hepatic mass was detected at 20 weeks' gestation. At birth (36 weeks), the hepatic mass measured 12x6 cm, and she had respiratory distress. Pulmonary hypertension (PHT) was detected on echocardiographic evaluation. Despite dual medical therapy, her PHT did not improve. Histologically, the biopsy demonstrated a mixed epithelial-mesenchymal HBL with predominance of fetal morphology in the epithelial component. Chemotherapy was initiated on postnatal day 15; however, the baby died of respiratory failure on postnatal day 23. Conclusion: HBL is an embryonal tumor which can develop early in the intrauterine period. Although the mechanism is not known, it may cause PHT which would affect the prognosis negatively.
