Background In the Japanese Study Group for Pediatric Liver Tumor (JPLT) protocols (JPLT-1 and 2) for evaluating the cure rate of risk-stratified hepatoblastoma, primary resection was permitted in PRETEXT I and II cases, followed by postoperative chemotherapy. Methods In approximately 500 enrolled cases, resection was performed as the initial treatment in 60 cases, including all 18 PRETEXT I, 30 PRETEXT II, and 12 ruptured cases. The clinical features, surgical procedures, complications, and survival rates were compared in these three groups. Results All 18 PRETEXT I cases underwent complete resection by lobectomy or segmentectomy (n = 14) or nonanatomical partial hepatectomy (NPH) (n = 4). The 30 PRETEXT II cases underwent primary resection by right or left lobectomy (n = 16), NPH (n = 10), or other procedures (n = 4). Of these 30 cases, operational death occurred in 1 newborn, and recurrence occurred in 7 cases (14.6%), including 6 NPH cases and 4 older cases (aged > 3 years). Of the 12 ruptured cases, 7 (58.3%) showed recurrence. Event-free survival rates at 5 years in the 3 groups were 88%, 70%, and 32%, respectively. Conclusions Primary resection for PRETEXT I or II HB cases should be performed by anatomical resection according to strict surgical guidelines. More intensified chemotherapy is required for primary resected cases whose tumors have ruptured.
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