[肾母细胞瘤——布拉格莫托尔大学医院30年的治疗历程]
[Nephroblastoma - 30-years period of its treatment in the University Hospital Motol, Prague].
作者信息
Mališ J, Švojgr K, Pýcha K, Jeřábková V, Cyprová S, Churáčková M, Šmelhaus V, Radvanský J, Zítková M, Kodet R, Kodetová D, Malinová B, Koutecký J, Šnajdauf J, Starý J
出版信息
Klin Onkol. 2013;26(5):336-42. doi: 10.14735/amko2013336.
INTRODUCTION
Nephroblastoma (Wilms tumor - WT) is the most common solid tumor of kidney in children. We present treatment development of WT at the Department of Pediatric Hematology and Oncology, Charles University in Prague, 2nd Faculty of Medicine and University Hospital Motol (KDHO) in the Czech Republic over 30 years. Patients that were treated prior to access to the International Society of Pediatric Oncology (SIOP) protocols are considered to be the historical group, then we have patients treated according to SIOP 9, SIOP 93-01 and SIOP 2001 protocols as full participants of SIOP studies.
PATIENTS AND METHODS
Between January 1980 and April 2009, we treated 330 patients with WT at KDHO: 91 patients in historical group (1980-1988), 94 pts in SIOP 9 (1988-1993), 80 pts in SIOP 93-01 (1994-2001) and 65 pts in SIOP 2001 (2002-2009). Overall survival (OS) and event-free survival (EFS) were analyzed by Kaplan-Meier test.
RESULTS
The overall ten-year EFS was 81.2% and OS 87.6%. Fifty-eight patients from the 330 (17.6%) had metastases at diagnosis, EFS without metastatic process was 84.6% compared to 65.4% with metastasis presented at diagnosis (p = 0.0003), OS was 70.7% compared to 91.2% (p < 0.0001). One hundred and seventy patients (51.5%) were treated with preoperative chemotherapy and/or radiotherapy, whereas 158 patients (47.5%) underwent primary nephrectomy; EFS and OS did not differ: neoadjuvant vs primary nephrectomy EFS was 81.2% vs 80.9% (p = 0.85), OS 89.4% vs 85.4% (p = 0.38). Sixty (18%) patients experienced disease recurrence; OS after relapse was 33%. In the historical group, EFS and OS were 85.7% and 91.2%. In patients treated according to the SIOP 9 protocol, EFS and OS were 68.1% and 74.5%, resp. In patients treated according to SIOP 93-01, it was 83.6% and 93.7%, resp. and in patients treated according to 87 SIOP 2001, it was 7% and 95.4% (p = 0.001 and p = 0.0008), resp.
CONCLUSION
WT is a well treatable disease. The aim for the future is to maintain the current very good survival while minimizing the treatment intensity.
引言
肾母细胞瘤（威尔姆斯瘤 - WT）是儿童最常见的肾脏实体瘤。我们展示了捷克共和国布拉格查理大学第二医学院和莫托尔大学医院（KDHO）儿科血液学和肿瘤学系30多年来肾母细胞瘤的治疗进展。在可获取国际儿科肿瘤学会（SIOP）方案之前接受治疗的患者被视为历史组，然后我们有根据SIOP 9、SIOP 93 - 01和SIOP 2001方案治疗的患者作为SIOP研究的正式参与者。
患者与方法
1980年1月至2009年4月期间，我们在KDHO治疗了330例肾母细胞瘤患者：历史组91例（1980 - 1988年），SIOP 9组94例（1988 - 1993年），SIOP 93 - 01组80例（1994 - 2001年），SIOP 2001组65例（2002 - 2009年）。通过Kaplan - Meier检验分析总生存（OS）和无事件生存（EFS）。
结果
总体十年EFS为81.2%，OS为87.6%。330例患者中有58例（17.6%）在诊断时已有转移，无转移过程的EFS为84.6%，而诊断时已有转移的为65.4%（p = 0.0003），OS分别为70.7%和91.2%（p < 0.0001）。170例患者（51.5%）接受了术前化疗和/或放疗，而158例患者（47.5%）接受了一期肾切除术；EFS和OS无差异：新辅助治疗与一期肾切除术相比，EFS分别为81.2%和80.9%（p = 0.85），OS分别为89.4%和85.4%（p = 0.38）。60例（18%）患者出现疾病复发；复发后的OS为33%。在历史组中，EFS和OS分别为85.7%和91.2%。根据SIOP 9方案治疗的患者，EFS和OS分别为68.1%和74.5%。根据SIOP 93 - 01方案治疗的患者，分别为83.6%和93.7%，根据SIOP 2001方案治疗的患者，分别为87%和95.4%（p = 0.001和p = 0.0008）。
结论
肾母细胞瘤是一种可良好治疗的疾病。未来的目标是在维持当前非常好的生存率的同时，尽量降低治疗强度。
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