Pretreatment prognostic factors and treatment results in children with hepatoblastoma -: A report from the German Cooperative Pediatric Liver Tumor Study HB 94
Pretreatment prognostic factors and treatment results in children with hepatoblastoma -: A report from the German Cooperative Pediatric Liver Tumor Study HB 94
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DOI：
10.1002/cncr.10632
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发表时间：
2002-07-01
期刊：
影响因子：
6.2
通讯作者：
Mildenberger, H
中科院分区：
文献类型：
--
作者：
Fuchs, J;Rydzynski, J;Mildenberger, H
背景。在过去的20年里，在多项国内和国际试验中，通过手术与化疗相结合，肝母细胞瘤（HB）患者的预后得到了显着改善。晚期或转移性 HB 患者的治疗仍然是一个世界范围内未解决的问题。方法。德国儿童肝脏肿瘤合作研究 HB 94 是一项前瞻性、多中心、单组研究。该研究从1994年1月持续到1998年12月。该方案评估了由顺铂、异环磷酰胺和阿霉素（CDDP/IFO/DOXO）和/或依托泊苷和卡铂（VP16/CARBO）组成的化疗的效率。分析手术策略、预处理因素和肿瘤特征对无病生存（DFS）的预后意义。结果。 HB 94 研究中治疗了 69 名 HB 儿童。幸存者的中位随访时间为 58 个月（范围 32-93 个月）。 69 名患者中有 53 名（77%）仍然活着，69 名患者中有 16 名（23%）死亡。长期DFS如下：27名患者中的26名为I期HB，3名患者中的3名为II期HB，25名患者中的19名为III期HB，14名患者中的5名为IV期。 63 名患者中有 54 名 (86%) 实现了原发肿瘤的完全切除。六名儿童（8%）未接受手术治疗。 22 名肿瘤被一期切除，41 名儿童在初步化疗后接受了手术。两名儿童接受了肝移植。无围手术期死亡。 48 名儿童接受了 CDDP/IFO/DOXO 初次化疗。 48 名儿童中有 41 名在 CDDP/IFO/DOXO 治疗后获得部分缓解。 18 名晚期或复发性 FIB 儿童接受了 VP16/CARBO 化疗，其中 12 名儿童获得缓解。治疗前相关的预后因素包括肝肿瘤的生长模式（P = 0.0135）、血管肿瘤侵犯（P = 0.0039）、远处转移的发生（P = 0.0001）、初始甲胎蛋白水平（P = 0.0034）和手术根治性（P < 0.0001）。结论。目前的结果强调了所有 FIB 儿童术前化疗的必要性。肿瘤完整切除是主要预后因素之一。 82. (C) 2002 年美国癌症协会。
BACKGROUND. in the past 20 years, a dramatic improvement in the prognosis of patients with hepatoblastoma (HB) has been achieved by combining surgery with chemotherapy in several national and international trials. A worldwide, unsolved problem remains the treatment of patients with advanced or metastatic HB.METHODS. The German Cooperative Pediatric Liver Tumor Study HB 94 was a prospective, multicenter, single-arm study. The study ran from January 1994 to December 1998. The protocol assessed the efficiency of chemotherapy consisting of cisplatin, ifosfamide, and doxorubicin (CDDP/IFO/DOXO) and/or etoposide and carboplatin (VP16/CARBO). The prognostic significance of the surgical strategy, pretreatment factors, and tumor characteristics for disease free survival (DFS) were analyzed.RESULTS. Sixty-nine children with HB were treated in the HB 94 study. The median follow-up of survivors was 58 months (range, 32-93 months). Fifty-three of 69 patients (77%) remained alive, and 16 of 69 patients (23%) died. Long-term DFS was as follows: 26 of 27 patients had Stage I HB, 3 of 3 patients had Stage II HB, 19 of 25 patients had Stage III HB, and 5 of 14 patients had Stage IV. A complete resection of the primary tumor was achieved in 54 of 63 patients (86%). Six children (8%) had no surgical treatment. Twenty-two tumors were resected primarily, and 41 children underwent surgery after initial chemotherapy. Two children underwent liver transplantation. There was no perioperative death. Forty-eight children received primary chemotherapy with CDDP/IFO/DOXO. Forty-one of 48 children achieved partial remission after CDDP/IFO/DOXO. Eighteen children with advanced or recurrent FIB underwent VP16/CARBO chemotherapy, with a response achieved by 12 children. The relevant pretreatment prognostic factors were growth pattern of the liver tumor (P = 0.0135), vascular tumor invasion (P = 0.0039), occurrence of distant metastases (P = 0.0001), initial a-fetoprotein level (P = 0.0034), and surgical radicality (P < 0.0001).CONCLUSIONS. The current results underline the necessity of preoperative chemotherapy in all children with FIB. Complete tumor resection is one of the main prognostic factors. 82. (C) 2002 American Cancer Society.