肾透明细胞肉瘤的治疗和结果：来自儿童肿瘤学组研究 AREN0321 和 AREN03B2 的报告。
Treatment and outcomes of clear cell sarcoma of the kidney: A report from the Children's Oncology Group studies AREN0321 and AREN03B2.
机构信息
Division of Hematology/Oncology, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Division of Biostatistics, University of Southern California, Los Angeles, California, USA.
出版信息
Cancer. 2024 Jul 1;130(13):2361-2371. doi: 10.1002/cncr.35266. Epub 2024 Feb 23.
BACKGROUND
On the fifth National Wilms Tumor Study, treatment for clear cell sarcoma of the kidney (CCSK) included combined vincristine, doxorubicin, cyclophosphamide, and etoposide (regimen I) plus radiation therapy (RT), yielding 5-year event-free survival (EFS) rates of 100%, 88%, 73%, and 29% for patients who had with stage I, II, III, and IV disease, respectively. In the Children's Oncology Group study AREN0321 of risk-adapted therapy, RT was omitted for stage I disease if lymph nodes were sampled, and carboplatin was added for stage IV disease (regimen UH-1). Patients who had stage II/III disease received regimen I with RT.
METHODS
Four-year EFS was analyzed for patients enrolled on AREN0321 and on those enrolled on AREN03B2 who received AREN0321 stage-appropriate chemotherapy.
RESULTS
Eighty-two patients with CCSK enrolled on AREN0321, 50 enrolled on AREN03B2 only. The 4-year EFS rate was 82.7% (95% confidence interval [CI], 74.8%-91.4%) for AREN0321 and 89.6% (95% CI, 81.3%-98.7%) for AREN03B2 only (p = .28). When combining studies, the 4-year EFS rates for patients who had stage I (n = 10), II (n = 47), III (n = 65), and IV (n = 10) disease were 90% (95% CI, 73.2%-100.0%), 93.4% (95% CI, 86.4%-100.0%), 82.8% (95% CI, 74.1%-92.6%), and 58.3% (95% CI, 34%-100.0%), respectively. There were no local recurrences among seven patients with stage I disease who were treated without RT. One stage I recurrence occurred in the brain, which was the most common site of relapse overall. Among patients with local stage III tumors, neither initial procedure type, margin status, nor lymph node involvement were prognostic.
CONCLUSIONS
Patients with stage I CCSK had excellent outcomes without local recurrences when treated without RT. Patients with stage IV disease appeared to benefit from a carboplatin-containing regimen, although their outcomes remained unsatisfactory. Further research is needed to improve outcomes for patients with advanced-stage disease (ClinicalTrials.gov identifiers NCT00335556 and NCT00898365).
背景
在第五届全国威尔姆斯肿瘤研究中，肾透明细胞肉瘤（CCSK）的治疗包括联合长春新碱、阿霉素、环磷酰胺和依托泊苷（方案 I）加放疗（RT），分别使 I 期、II 期、III 期和 IV 期疾病患者的 5 年无事件生存率（EFS）达到 100%、88%、73%和 29%。在儿童肿瘤学组 AREN0321 的风险适应治疗研究中，如果淋巴结取样，则省略 I 期疾病的 RT，并为 IV 期疾病（方案 UH-1）添加卡铂。II/III 期疾病患者接受 RT 的方案 I。
方法
对接受 AREN0321 治疗的患者和仅接受 AREN03B2 治疗的患者进行了 4 年 EFS 分析。
结果
共有 82 例 CCSK 患者接受了 AREN0321 治疗，50 例患者仅接受了 AREN03B2 治疗。AREN0321 的 4 年 EFS 率为 82.7%（95%CI，74.8%-91.4%），而仅接受 AREN03B2 治疗的患者为 89.6%（95%CI，81.3%-98.7%）（p=0.28）。将研究合并后，I 期（n=10）、II 期（n=47）、III 期（n=65）和 IV 期（n=10）疾病患者的 4 年 EFS 率分别为 90%（95%CI，73.2%-100.0%）、93.4%（95%CI，86.4%-100.0%）、82.8%（95%CI，74.1%-92.6%）和 58.3%（95%CI，34%-100.0%）。7 例 I 期患者未接受 RT 治疗，均无局部复发。1 例 I 期复发发生在脑部，这是总体上最常见的复发部位。对于局部 III 期肿瘤患者，初始手术类型、切缘状态和淋巴结受累均与预后无关。
结论
未接受 RT 治疗的 I 期 CCSK 患者无局部复发，预后良好。IV 期疾病患者似乎从含卡铂的方案中受益，但他们的结局仍不理想。需要进一步研究以改善晚期疾病患者的预后（ClinicalTrials.gov 标识符 NCT00335556 和 NCT00898365）。
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