Outcome of patients with a first relapse after intermediate- or high-risk Wilms tumor, treated according to SIOP WT 2001/UK-IMPORT study; A report from the SIOP renal tumor study group.

IF 2.3 3区医学 Q3 ONCOLOGY

Urologic Oncology-seminars and Original Investigations Pub Date : 2025-06-21 DOI:10.1016/j.urolonc.2025.05.018

Alissa Groenendijk, Harm van Tinteren, Ronald R de Krijger, Gordan Vujanic, Reem Al-Saadi, Kathy Pritchard-Jones, Gema L Ramírez-Villar, Patrick Melchior, Jan Godzinski, Jens-Peter Schenk, Norbert Graf, Heidi Segers, Hélène Sudour-Bonnange, Arnauld C Verschuur, Jarno Drost, Daniela Perotti, Sarah Al-Jilaihawi, Jesper Brok, Marry M van den Heuvel-Eibrink, Filippo Spreafico, Annelies M C Mavinkurve-Groothuis

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引用次数: 0

Abstract

Background: Patients with a Wilms tumor (WT) who relapse following initial therapy with more than only vincristine and actinomycin-D are considered high-risk (group BB) or very high-risk (group CC) relapse by the International Society of Pediatric Oncology - Renal Tumor Study Group (SIOP-RTSG). We aimed to retrospectively analyze the characteristics and outcome of BB and CC patients.

Methods: We included all patients with first relapsed WT that would currently be considered BB (n = 148) and CC (n = 72) relapse and registered in the SIOP 2001/UK-IMPORT study. We collected information on relapse treatment and calculated 5-year event-free (EFS) and overall survival (OS) rates per relapse risk group and treatment. Multivariable Cox regression analysis was performed to identify patient and tumor characteristics that were significantly associated with survival.

Findings: The 5-year estimated EFS and OS rates of BB patients were 62·7% (95% CI: 54·9-71·6%) and 67·6% (95% CI: 59·8-76·4%), respectively. Five-year survival rates for the subset of BB patients treated with VAD and RT (n = 42/94) were 58·7% (95% CI: 45·0-76·6%) for EFS and 66·5% (95% CI: 53·1-83·2%) for OS. Five-year estimated EFS and rates for CC patients were 17·4% (95% CI: 10·4-29·0%) and 18·6% (95% CI: 11·3-30·5%), respectively. In multivariable Cox regression analysis, patients seemed to benefit from high dose chemotherapy and autologous stem cell rescue (HDCT) and surgery at first relapse.

Interpretation: Second-line treatment was able to rescue two-thirds of BB relapse patients. In contrast, survival rates in CC patients at first relapse remain poor with conventional drugs, even with camptothecin-containing regimens. A subset of BB and CC patients may benefit from HDCT and surgery at relapse. However, to validate these findings, they must be reevaluated in future trials to eliminate bias from the analyses that is inherent to retrospective studies.

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根据SIOP WT 2001/UK-IMPORT研究，治疗中高风险Wilms肿瘤后首次复发患者的预后来自SIOP肾肿瘤研究组的报告。

背景：国际儿科肿瘤学会-肾肿瘤研究组（sip - rtsg）认为，Wilms肿瘤（WT）患者在初始治疗中使用超过长春新碱和放线菌素- d治疗后复发为高危（BB组）或高危（CC组）复发。我们旨在回顾性分析BB和CC患者的特征和预后。方法：我们纳入了所有首次复发的WT患者，目前被认为是BB （n = 148）和CC （n = 72）复发，并登记在SIOP 2001/UK-IMPORT研究中。我们收集了有关复发治疗的信息，并计算了每个复发风险组和治疗的5年无事件（EFS）和总生存率（OS）。进行多变量Cox回归分析，以确定与生存显著相关的患者和肿瘤特征。结果：BB患者的5年估计EFS和OS率分别为62.7% （95% CI: 54.9 - 71.6%）和67.6% （95% CI: 59.8 - 76.4%）。接受VAD和RT治疗的BB患者亚群的5年生存率（n = 42/94）为EFS的58.7% (95% CI: 45.0 ~ 76.6%)， OS的66.5% （95% CI: 53.1 ~ 83.2%）。CC患者的5年估计EFS和发生率分别为17.4% （95% CI: 10.4 - 29.0%）和18.6% （95% CI: 11.3 - 30.5%）。在多变量Cox回归分析中，患者在首次复发时似乎受益于高剂量化疗、自体干细胞抢救（HDCT）和手术。解释：二线治疗能够挽救三分之二的BB复发患者。相比之下，在首次复发的CC患者中，即使使用含喜树碱的治疗方案，生存率仍然很低。一部分BB和CC患者复发时可以通过HDCT和手术获益。然而，为了验证这些发现，它们必须在未来的试验中重新评估，以消除回顾性研究固有的分析偏差。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Urologic Oncology-seminars and Original Investigations 医学-泌尿学与肾脏学

CiteScore

4.80

自引率

3.70%

发文量

297

审稿时长

7.6 weeks

期刊介绍： Urologic Oncology: Seminars and Original Investigations is the official journal of the Society of Urologic Oncology. The journal publishes practical, timely, and relevant clinical and basic science research articles which address any aspect of urologic oncology. Each issue comprises original research, news and topics, survey articles providing short commentaries on other important articles in the urologic oncology literature, and reviews including an in-depth Seminar examining a specific clinical dilemma. The journal periodically publishes supplement issues devoted to areas of current interest to the urologic oncology community. Articles published are of interest to researchers and the clinicians involved in the practice of urologic oncology including urologists, oncologists, and radiologists.