Outcome of pediatric renal tumor treated using the Japan Wilms Tumor Study-1 (JWiTS-1) protocol: a report from the JWiTS Group
Outcome of pediatric renal tumor treated using the Japan Wilms Tumor Study-1 (JWiTS-1) protocol: a report from the JWiTS Group
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DOI：
10.1007/s00383-009-2449-0
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发表时间：
2009-11-01
影响因子：
1.8
通讯作者：
Tanaka, Yukichi
中科院分区：
文献类型：
--
作者：
Oue, Takaharu;Fukuzawa, Masahiro;Tanaka, Yukichi
1996年，日本Wilms肿瘤研究（JWiTS）小组成立，以阐明美国国家Wilms肿瘤研究（NWTS）小组建立的方案的有效性和安全性，日本也开始了多中心合作研究（JWiTS-1）。本报告回顾了JWiTS-1的结果。1996年至2005年间，共有307例恶性肾肿瘤患者被纳入JWiTS-1研究。210例获得中心病理诊断及随访资料。该方案方案与NWTS-5方案相似。临床分期按日本分期法进行。肾母细胞瘤的5年总生存率（OS）为91.1%，肾透明细胞肉瘤（CCSK）为72.9%，肾横纹肌样瘤（RTK）为22.2%。在肾母细胞瘤患者中，I期患者的5年OS为90.5%，II期为92.2%，III期为90.9%，IV期为86.7%，v期为78.7%。JWiTS-1研究中患者的OS与美国和欧洲其他多中心研究的结果相当。肾母细胞瘤和CCSK患者的结果是公平的。相比之下，RTK患者的治愈率并不令人满意。RTK患者需要新的治疗策略。
In 1996, the Japan Wilms Tumor Study (JWiTS) group was founded to elucidate the efficacy and safety of the regimen established by the National Wilms Tumor Study (NWTS) group in the USA, and a multicenter cooperative study (JWiTS-1) was started in Japan. This report reviews the results of JWiTS-1.A total of 307 patients with malignant renal tumor were enrolled in the JWiTS-1 study between 1996 and 2005. Central pathological diagnosis and follow-up data were available in 210 cases. The protocol regimens were similar to the NWTS-5 regimens. Clinical stage was classified according to the Japanese Staging System.Five-year overall survival (OS) rate was 91.1% for nephroblastoma, 72.9% for clear cell sarcoma of the kidney (CCSK), and 22.2% for rhabdoid tumor of the kidney (RTK). In the nephroblastoma patients, 5-year OS was 90.5% for stage I disease, 92.2% for stage II, 90.9% for stage III, 86.7% for stage IV, and 78.7% for stage V.The OS of patients in the JWiTS-1 study were comparable with the results of other multicenter studies in the USA and Europe. The outcome for patients with nephroblastoma and CCSK was fair. In contrast, the cure rate for those with RTK was not satisfactory. New treatment strategies are needed for patients with RTK.