Grundy R G, Hutton C, Middleton H, Imeson J, Pritchard J, Kelsey A, Marsden H B, Vujanic G M, Taylor R E
Institute of Child Health, University of Birmingham, Whittall Street Birmingham, United Kingdom.
Pediatr Blood Cancer. 2004 Apr;42(4):311-9. doi: 10.1002/pbc.10477.
The aims of UKWT2 included consolidating the results for stage III patients obtained in UKWT1 and improving the outcome for patients with inoperable tumours by giving vincristine, actinomycin-D and doxorubicin in an intensive schedule (Intensive AVA).
The second UK WT trial (UKWT2) ran between July 1986 and September 1991 accruing 448 patients. One hundred and six patients were diagnosed and treated for stage III disease. Six had clear cell sarcoma of the kidney (CCSK) and seven had rhabdoid tumours of the kidney (RTK) and are analysed separately. One other patient was excluded from overall analysis. Ninety-two patients were followed for a median of 115 months. Seventy-five received standard chemotherapy and abdominal radiotherapy according to protocol. Seventeen had stage III disease at immediate nephrectomy, but radiotherapy was omitted by physician choice. Thirty-three patients had inoperable disease at diagnosis and received pre-nephrectomy chemotherapy.
Overall survival (OS) at 4 years for stage III favourable histology (FH) patients receiving abdominal RT was 83% (CI: 73-89). For children with stage III disease in whom RT was omitted the OS was 82% (CI: 59-97) and for inoperable disease 94% (CI: 78-98). The overall and event-free survival (EFS) of children with stage III CCSK was 100% and was achieved with the majority of patients not receiving radiotherapy (CI: 48-100). Three of seven children with RTK are alive EFS and OS 43% (CI: 10-73). For patients treated by abdominal radiotherapy the overall local control rate was 94.4% (CI: 86.4-98.5*%), 96.7% (CI: 88.5-99.6%) for flank RT and 83.3% (51.6-98.0%) for whole abdominal radiotherapy (WRT).
The outcome for stage III FH disease was similar to that reported for UKWT1 and NWTS-3. The combination of abdominal RT together with 3-drug chemotherapy achieves a high abdominal tumour control rate. Flank RT is probably sufficient for localised tumour rupture. The high cure rates for children in this trial with 'inoperable disease' suggests that treatment should be modified according to their post-chemotherapy stage in order to avoid over-treatment. The high OS for stage III CCSK on this protocol suggests that treatment duration could be curtailed and the role of RT reviewed, though the numbers are small. The prognosis for older children with RTK seems to be better than for younger children although larger studies are required to confirm this.
UKWT2的目的包括巩固UKWT1中获得的III期患者的结果，并通过密集方案（强化AVA）给予长春新碱、放线菌素-D和阿霉素来改善不可切除肿瘤患者的预后。
第二项英国肾母细胞瘤试验（UKWT2）于1986年7月至1991年9月进行，招募了448名患者。106名患者被诊断为III期疾病并接受治疗。6例患有肾透明细胞肉瘤（CCSK），7例患有肾横纹肌样瘤（RTK），分别进行分析。另有1例患者被排除在总体分析之外。92例患者的中位随访时间为115个月。75例患者按照方案接受了标准化疗和腹部放疗。17例在即刻肾切除时患有III期疾病，但医生选择省略了放疗。33例患者在诊断时患有不可切除疾病，并接受了肾切除术前化疗。
接受腹部放疗的III期组织学良好（FH）患者4年总生存率（OS）为83%（CI：73 - 89）。对于省略放疗的III期疾病儿童，OS为82%（CI：59 - 97），对于不可切除疾病为94%（CI：78 - 98）。III期CCSK儿童的总生存率和无事件生存率（EFS）为100%，大多数患者未接受放疗（CI：48 - 100）。7例RTK儿童中有3例存活，EFS和OS为（CI：10 - 73）。对于接受腹部放疗的患者，总体局部控制率为94.4%（CI：86. .4 - 98.5*%），侧腹放疗为96.7%（CI：88.5 - 99.6%），全腹放疗（WRT）为83.3%（51.6 - 9 .80%）。
III期FH疾病的结果与UKWT1和NWTS - 3报告的结果相似。腹部放疗与三联化疗联合可实现较高的腹部肿瘤控制率。侧腹放疗可能足以控制局部肿瘤破裂。本试验中“不可切除疾病”儿童的高治愈率表明，应根据化疗后阶段调整治疗，以避免过度治疗。该方案中III期CCSK的高OS表明，尽管病例数较少，但可以缩短治疗时间并重新评估放疗的作用。年龄较大的RTK儿童的预后似乎比年龄较小的儿童好，尽管需要更大规模的研究来证实这一点。
