Liver resection extended to adjacent structures for advanced hepatoblastoma - a 25 year tertiary hepatobiliary and transplant center experience.
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BACKGROUND: Refinement in perioperative chemotherapy coupled with surgical innovation improves prognosis in children with very high risk hepatoblastoma. Our aim was to evaluate and identify prognostic factors contributing to recurrence in hepatoblastoma resected along with adjacent structures. METHODS: An audit was conducted of patients surgically treated for hepatoblastoma at our center over 25 years. RESULTS: Thirty-six of 202 patients underwent resection of structures adjacent to the liver for suspected tumor spread. Over half (21/36) of patients underwent hepatectomy with resection of adjacent structures, and orthotopic liver or multi-visceral transplantation. Adjacent structures including lymph nodes, vascular structures, diaphragm, spleen, omentum, and stomach, showed viable tumor tissue in nineteen patients. Both overall survival and recurrence free survival were 75 % at a mean follow up of 113 months. Survival improved with a negative resection margin. Recurrence free survival decreased with tumor viability. Pathology subtypes showed distinct influence on survival. CONCLUSION: This series shows favorable survival with aggressive surgical treatment. Adverse histology subtype, lung metastases, and resistance to chemotherapy are associated with higher risk of recurrence. Management at specialist centers with simultaneous hepatic resection and transplantation readily available, achieves optimum outcomes in this niche subgroup of children with advanced hepatoblastoma.