[上海儿童医学中心肾母细胞瘤试验（WT-99）的结果]

Tang Jing-yan, Pan Ci, Xu Min, Xue Hui-liang, Chen Jing, Zhao Hui-lun, Gu Long-lun, Wang Yao-ping

Department of Hematology/Oncology, XinHua Hospital/Shanghai Children's Medical Center, Shanghai Second Medical University, Shanghai 200127, China.

Zhonghua Er Ke Za Zhi. 2003 Feb;41(2):131-4.

OBJECTIVE

Wilms' Tumor Trial (WT-99) of Shanghai Children's Medical Center was designed and conducted by applying therapeutic regimens stratified by stage and histology in accordance with National Wilms' Tumor Study (NWTS) criteria of U.S.A. The main aim of WT-99 was to reduce treatment of low-stage, favorable-histology (FH) tumors without impairing survival and to improve prognosis of stage III and IV (FH) and unfavorable-histology (UFH) tremors with more intensive chemotherapy.

METHODS

Diagnosis and treatment was decided by the multi-disciplinary team including oncologists, surgeons, pathologists, radiologists and diagnostic radiologists. Twenty consecutively diagnosed patients were recruited between October 1998 and October 2002. The regimen for patients at favorable-histology (FH) stage I and II and anaplastic stage I was vincristine (Vcr) and dactinomycin (Act-D) only, while for those at focal anaplastic stage II to IV and FH stage III and IV the regimen was Vcr, Act-D and adriamycin (Adr). Patients at diffuse anaplastic stage II to IV and clear cell stage I to IV received four-drug regimen including Vcr, etoposide (VP16), Adr and cytoxan (CTX). For those at rhabdoid stage I to IV the regimen was carboplatin, VP-16 and CTX. Un-resectable patients received 2 courses of Ifosfamide, Vcr and VP-16 as pre-surgery therapy. No radiation therapy was used for patients at stage I and FH stage II.

RESULTS

Twenty patients, from 7 months to 12 years old, were enrolled. Pathologic analysis showed fourteen cases were at their FH, three at unfavorable-histology (UFH), two at clear cell and one at rhabdoid stage. Five patients were at stage I, five at stage II, six at stage III, three at stage IV and one at stage V. Eighteen reached complete response (90%), and two failed. One relapsed after 24 months of CCR and reached the second CR after intensive chemotherapy. No therapy-related death happened. Survival rate (SR) was 90% (18/20) and event-free survival (EFS) was 85% (17/20) at 11-45 months, average 27 months.

CONCLUSION

Multi-disciplinary team work model and protocol WT-99 are safe and effective for Wilms' tumor.

目的

上海儿童医学中心的肾母细胞瘤试验（WT - 99）是按照美国国家肾母细胞瘤研究（NWTS）标准，应用根据分期和组织学分层的治疗方案设计并开展的。WT - 99的主要目的是在不影响生存率的情况下减少低分期、组织学良好（FH）肿瘤的治疗，并通过更强化的化疗改善Ⅲ期和Ⅳ期（FH）以及组织学不良（UFH）肿瘤的预后。

方法

诊断和治疗由包括肿瘤学家、外科医生、病理学家、放射科医生和诊断放射科医生在内的多学科团队决定。1998年10月至2002年10月期间连续招募了20例确诊患者。组织学良好（FH）的Ⅰ期和Ⅱ期以及间变Ⅰ期患者的治疗方案仅为长春新碱（Vcr）和放线菌素D（Act - D），而局灶性间变Ⅱ期至Ⅳ期以及FH的Ⅲ期和Ⅳ期患者的治疗方案为Vcr、Act - D和阿霉素（Adr）。弥漫性间变Ⅱ期至Ⅳ期以及透明细胞Ⅰ期至Ⅳ期患者接受包括Vcr、依托泊苷（VP16）、Adr和环磷酰胺（CTX）的四联方案。横纹肌样Ⅰ期至Ⅳ期患者的治疗方案为卡铂、VP - 16和CTX。无法切除的患者接受2个疗程的异环磷酰胺、Vcr和VP - 16作为术前治疗。Ⅰ期和FH的Ⅱ期患者未使用放射治疗。

结果

招募了20例年龄从7个月至12岁的患者。病理分析显示，14例为FH，3例为组织学不良（UFH），2例为透明细胞，1例为横纹肌样期。5例为Ⅰ期，5例为Ⅱ期，6例为Ⅲ期，3例为Ⅳ期，1例为Ⅴ期。18例达到完全缓解（90%），2例未缓解。1例在持续完全缓解24个月后复发，经强化化疗后达到第二次完全缓解。未发生与治疗相关的死亡。在11 - 45个月（平均27个月）时，生存率（SR）为90%（18/20），无事件生存率（EFS）为85%（17/20）。