Outcome of renal tumors registered in Japan Wilms Tumor Study-2 (JWiTS-2): A report from the Japan Children's Cancer Group (JCCG)

Outcome of renal tumors registered in Japan Wilms Tumor Study-2 (JWiTS-2): A report from the Japan Children's Cancer Group (JCCG)
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DOI:
10.1002/pbc.27056
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发表时间:
2018-07-01
影响因子:
3.2
通讯作者:
Fukuzawa, Masahiro
Fukuzawa, Masahiro
中科院分区:
医学3区
文献类型:
--
作者:
Koshinaga, Tsugumichi;Takimoto, Tetsuya;Fukuzawa, Masahiro

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背景日本肾母细胞瘤研究 2 (JWiTS-2) 要求对所有病例登记进行中央病理学审查。该研究旨在比较参加 JWiTS-1 和 JWiTS-2 试验的单侧肾母细胞瘤患者的结果。 程序 JWiTS-2 试验(2006-2014)是一项前瞻性、单臂研究,要求强制向中心病理学提交组织学切片,而在 JWiTS-1 试验中,这种提交不是强制性的。对 JWiTS-1 试验(1996-2005)中病例的无复发生存率(RFS)和总生存率(OS)进行统计评估。结果在 277 名经中央病理审查系统诊断的原发性肾肿瘤入组患者中,其中 225 名单侧肾肿瘤患者接受了超过 9 年的随访。 Wilms 肿瘤 (n=178) 的 RFS 和 OS 分别为 90.4% (P=0.0003) 和 96.8% (P=0.054),而 JWiTS-1 的 RFS 和 OS 分别为 74.9% 和 89.4%。 JWiTS-2中I-III期Wilms肿瘤的RFS率超过90%,尽管IV期Wilms肿瘤的结果明显较差(RFS:66.2%)(P=0.0094)。肾透明细胞肉瘤(CCSK;n=31)的 RFS 和 OS 分别为 82.4%(P=0.30)和 91.3%(P=0.42),而 JWiTS-1 为 68.8% 和 81.3%;肾横纹肌瘤(RTK;n=16)的 RFS 和 OS 分别为 18.8%(P=0.88)和分别为 25.0% (P=0.80),而 JWiTS-1 为 23.5% 和 23.5%。结论与 JWiTS-1 相比,JWiTS-2 中 I-III 期 Wilms 肿瘤的 RFS 和 OS 得到改善,而 IV 期 Wilms 肿瘤、CCSK 和 RTK 的结果没有改善。
BackgroundJapan Wilms Tumor Study-2 (JWiTS-2) mandated central pathology review for all case registrations. The study aimed to compare the outcomes of patients with unilateral Wilms tumor enrolled on the JWiTS-1 and JWiTS-2 trials.ProcedureThe JWiTS-2 trial (2006-2014), a prospective, single-arm study, required compulsory submission of histologic slides to central pathology, while in the JWiTS-1 trial, such submission was not compulsory. Relapse-free survival (RFS) and overall survival (OS) versus cases in the JWiTS-1 trial (1996-2005) were statistically evaluated.ResultsOf 277 enrolled patients with primary renal tumors diagnosed by the central pathology review system, 225 patients with unilateral renal tumors were followed up over 9 years. The RFS and OS of Wilms tumor (n=178) were 90.4% (P=0.0003) and 96.8% (P=0.054), respectively, as compared to 74.9% and 89.4% in JWiTS-1. RFS rates of stages I-III Wilms tumor in JWiTS-2 were more than 90%, although the outcome of stage IV Wilms tumor was significantly poorer (RFS: 66.2%) (P=0.0094). RFS and OS of clear cell sarcoma of the kidney (CCSK; n=31) were 82.4% (P=0.30) and 91.3% (P=0.42), respectively, as compared to 68.8% and 81.3% in JWiTS-1, and those of rhabdoid tumor of the kidney (RTK; n=16) were 18.8% (P=0.88) and 25.0% (P=0.80), respectively, as compared to 23.5% and 23.5% in JWiTS-1.ConclusionsRFS and OS for stages I-III Wilms tumor were improved in JWiTS-2 compared to JWiTS-1, whereas outcomes for stage IV Wilms tumor, CCSK, and RTK did not improve.