Abstract

Primary liver transplantation is recommended for central post-treatment extent of disease (POST-TEXT) III and IV hepatoblastoma. The aim of this study was to prospectively assess the safety and oncological efficacy of aggressive non-transplant extended hepatic resection in these patients. A prospective study involved 18 children with central pretreatment extent of disease (PRETEXT) III and IV: three had primary liver transplantation whereas 15 underwent hepatic resection after neoadjuvant chemotherapy. Median tumor volume was 317 mL (range, 135-546 mL). After four cycles of chemotherapy, POST-TEXT stage was III in 12 patients and IV in three patients. There was no perioperative mortality. Postoperative complications consisted of two bile leaks, one temporary decompensation and one sub-phrenic collection requiring drainage. One and 3year disease-free survival was 93.3% and 73.3% respectively. The 3year overall survival was 86.6%. Four patients developed recurrence, of whom two died. Early recurrence in 1year occurred in one patient. All recurrences were distant metastases. Extended major hepatic resection for selected cases of POST-TEXT III and IV hepatoblastoma is a technically challenging but feasible approach with acceptable morbidity and mortality rates. Oncological outcomes are similar to liver transplantation without the long-term commitment of immunosuppression or donor risk and morbidity, but a potential donor should always be organized on standby.

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