Outcome of hepatoblastomas treated using the Japanese Study Group for Pediatric Liver Tumor (JPLT) protocol-2: report from the JPLT
Outcome of hepatoblastomas treated using the Japanese Study Group for Pediatric Liver Tumor (JPLT) protocol-2: report from the JPLT
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DOI：
10.1007/s00383-010-2708-0
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发表时间：
2011-01-01
影响因子：
1.8
通讯作者：
Hiyama, Eiso
中科院分区：
文献类型：
--
作者：
Hishiki, Tomoro;Matsunaga, Tadashi;Hiyama, Eiso
背景近年来，肝母细胞瘤手术切除加术前和/或术后化疗显著提高了患者的生存率。我们在此报告了日本儿童肝肿瘤研究组（JPLT-2）现行方案治疗患者的结果。方法共有279例恶性肝肿瘤患者入选JPLT-2。本文分析了212例肝母细胞瘤的临床资料。PRETEXT I患者接受了原发性切除术，随后接受低剂量顺铂-吡拉西坦（四氢吡喃阿霉素）治疗。否则，患者术前接受顺铂-吡拉托（CITA），随后接受手术和术后化疗。给予异环磷酰胺、吡拉西坦、依托泊苷和卡铂（ITEC）作为挽救治疗。结果非转移患者的5年总生存率（OS）：PRETEXT I组为100%，PRETEXT II组为87.1%，PRETEXT III组为89.7%，PRETEXT IV组为78.3%。转移性病例的5年OS为43.9%。非转移性PRETEXT IV病例的结局明显改善，而转移性肿瘤的结果仍然poor.Conclusions JPLT-2方案取得了令人满意的生存率在儿童非转移性肝母细胞瘤。转移性疾病患者需要新的治疗方法。
Background In the recent years, surgical resection with pre-and/or postoperative chemotherapy has markedly improved the survival rate of hepatoblastoma patients. We herein report the results of patients treated with the current protocol of the Japanese Study Group for Pediatric Liver Tumor, JPLT-2.Methods A total of 279 patients with malignant liver tumor were enrolled in JPLT-2. Data from 212 hepatoblastoma cases were analyzed. PRETEXT I patients were treated with primary resection followed by low doses of cisplatin-pirarubicin (tetrahydropyranyl-adriamycin). Otherwise, patients received preoperative cisplatin-pirarubicin (CITA), followed by surgery and postoperative chemotherapy. Ifosfamide, pirarubicin, etoposide, and carboplatin (ITEC) were given as a salvage treatment. High-dose chemotherapy with hematopoietic stem cell transplantation (SCT) was reserved for patients with metastatic diseases.Results The 5-year overall survival rate (OS) in non-metastatic cases was 100% for PRETEXT I, 87.1% for PRETEXT II, 89.7% for PRETEXT III, and 78.3% for PRETEXT IV. The 5-year OS in metastatic cases was 43.9%. The outcome in non-metastatic PRETEXT IV cases was markedly improved, while the results of metastatic tumors remained poor.Conclusions JPLT-2 protocol achieved satisfactory survival among children with non-metastatic hepatoblastoma. New approaches are needed for patients with metastatic diseases.