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Introduction: Hepatoblastoma is the most common malignant primary liver tumor in the pediatric population, accounting for 67% of cases in the United States. Surgical resection is the only curative treatment option; however, it can be performed in only 10% of patients with primary tumors. The two most common limitations for resection are the need for extensive resections and tumors in central locations. The therapeutic hypertrophy of healthy tissue achieved with ALPPS (Associating Liver Partition and Portal vein ligation for Staged Hepatectomy) enables larger resections and has been successfully employed in the pediatric population in recent years. Objective: To present three cases of patients with centrally located PRETEXT II or III hepatoblastomas who underwent ALPPS procedure as a viable therapeutic alternative to liver transplantation. Discussion and results: Central PRETEXT III hepatoblastomas are typically indications for liver transplantation. Transplantation offers high five-year survival rates (73%). However, the associated morbidity, healthcare system costs, and limited availability make it necessary to explore alternative options. Series have reported the successful application of the ALPPS procedure in PRETEXT II and PRETEXT III hepatoblastomas in other locations. Therapeutically induced hypertrophy, characterized by an increase in the volume of healthy tissue in unaffected lobes or segments, enabled the resection of previously deemed unresectable lesions. The patients experienced uncomplicated postoperative courses and expected reduction in tumor markers. Chemotherapy selection followed the guidelines outlined in Block C of the SIOPEL IV protocol. Conclusions: ALPPS hepatectomy is a viable therapeutic option for patients with centrally located PRETEXT III or II hepatoblastomas.
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INTRODUCTION: Congenital diaphragmatic hernias are a rare developmental defect. Pulmonary complications are more frequently seen in right sided defects (Partridge et al., 2016). Hepatopulmonary fusion is a rare and highly mortal malformation exclusively seen in right sided congenital diaphragmatic hernias marked by the fibrovascular fusion of the liver and lung. PRESENTATION OF CASE: A newborn male presented with respiratory distress and a 1-minute APGAR score of 7. A chest radiograph showed complete opacification of the right hemithorax, and a CT-Scan confirmed a congenital diaphragmatic hernia, an intrathoracic location of the liver and secondary pulmonary hypoplasia. 48 h after, intraoperative findings showed fusion of diaphragm, lung and liver tissue. Four months after, complete tissue division of the lower lobe from the fused liver segments VII/VIII and correction of the hernia defect was achieved. The patient was discharged from the hospital six months after. DISCUSSION: Partial division of tissues is described as the safest and most successful approach to hepatopulmonary fusion. The tally of all cases reported worldwide until 2020 shows higher survival rates with complete division of tissues (Ferguson DM; Congenital Diaphragmatic Hernia Study Group, 2020) Reported cases lean towards one-session surgical interventions. A two-stage surgical approach allows an initial low surgical trauma to manage compressive effects on intrathoracic structures by herniary contents and a second time for tissue division in a non-critical patient, in this case leading to long-term survival. CONCLUSION: Hepatopulmonary fusion is a rare and highly lethal malformation with scarce information available. Future multicenter studies should compare different therapeutic options and search for outcomes including but not limited to mortality.
