III 期组织学有利的肾母细胞瘤中淋巴结受累和 1p 或 16q 杂合性丧失的预后影响：儿童肿瘤小组研究 AREN03B2 和 AREN0532 的报告。
Prognostic impact of lymph node involvement and loss of heterozygosity of 1p or 16q in stage III favorable histology Wilms tumor: A report from Children's Oncology Group Studies AREN03B2 and AREN0532.
发表日期：2023 Oct 30
作者：
Nicholas Evageliou, Lindsay A Renfro, James Geller, Elizabeth Perlman, John Kalapurakal, Arnold Paulino, David Dix, Meryle J Eklund, Andrew J Murphy, Rodrigo L P Romao, Peter F Ehrlich, Carly R Varela, Kelly Vallance, Conrad V Fernandez, Jeffrey S Dome, Elizabeth A Mullen
来源：
CANCER
摘要：
在仅参加 AREN0532 或 AREN03B2 的 III 期组织学良好肾母细胞瘤 (FHWT) 儿童中，评估了阳性淋巴结 (LN ) 和/或 1p 或 16q 单一杂合性缺失 (LOH) 的预后影响。总共 635 名分期患者III FHWT 长春新碱/放线菌素/阿霉素 (DD4A) 治疗的患者符合纳入标准。无事件生存期 (EFS) 和总生存期通过 LN 采样、LN 状态、LOH 1p、LOH 16q 以及 LN 状态和单个 LOH 的组合来报告。排除 1p 和 16q 状态未知或阳性组合 LOH 的患者以及结果未知或除 DD4A 以外治疗的仅 AREN03B2 患者。EFS 在研究中没有差异，支持合并。缺乏 LN 采样（风险比 [HR]，2.12；p = .0037）、LN 阳性（HR，2.78；p = .0002）、LOH 1p（HR，2.18；p = .0067）和 LOH 16q（HR） , 1.72; p = .042) 与较差的 EFS 相关。与同时患有 LN- 和 LOH- 的患者相比，淋巴结阴性但 LOH 1p 或 16q 阳性的患者以及具有 LN 但 1p 或 16q LOH- 的患者的 EFS 显着较差（HR 分别为 3.05 和 3.57）。 LN 和 LOH 均呈阳性的患者的 EFS 最差（HR，6.33；总体组因素，p < .0001）。研究结果证实，LN 状态是一个不良预后因素，因单个 LOH 1p 或 16q 的存在而放大，支持强化治疗的研究在一项前瞻性临床试验中，针对 LN 合并单一 LOH 的患者。© 2023 美国癌症协会。
The prognostic impact of positive lymph nodes (LN+) and/or singular loss of heterozygosity (LOH) of 1p or 16q were assessed in children with stage III favorable histology Wilms tumor (FHWT) enrolled on AREN0532 or AREN03B2 alone.A total of 635 stage III FHWT vincristine/dactinomycin/doxorubicin (DD4A)-treated patients met inclusion criteria. Event-free survival (EFS) and overall survival are reported overall and by LN sampling, LN status, LOH 1p, LOH 16q, and a combination of LN status and singular LOH. Patients with unknown or positive combined LOH of 1p and 16q status and AREN03B2-only patients with unknown outcomes or treatment other than DD4A were excluded.EFS did not differ by study, supporting pooling. Lack of LN sampling (hazard ratio [HR], 2.12; p = .0037), LN positivity (HR, 2.78; p = .0002), LOH 1p (HR, 2.18; p = .0067), and LOH 16q (HR, 1.72; p = .042) were associated with worse EFS. Compared with patients with both LN- and LOH-, those with negative nodes but positive LOH 1p or 16q and those with LN+ but LOH- for 1p or 16q had significantly worse EFS (HR, 3.05 and 3.57, respectively). Patients positive for both LN and LOH had the worst EFS (HR, 6.33; overall group factor, p < .0001).Findings confirm LN+ status as an adverse prognostic factor amplified by presence of singular LOH 1p or 16q, supporting study of intensified therapy for patients with LN+ in combination with singular LOH in a prospective clinical trial.© 2023 American Cancer Society.