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威尔姆斯瘤(肾母细胞瘤)的生存特征,来源于一项纵向队列研究的参考资料。

Survival characteristics of Wilms Tumor, a reference developed from a longitudinal cohort study.

机构信息

Faculty of Medicine, Cairo University, Cairo, Egypt.

Department of Surgery, University of Toledo Medical Center, Toledo, OH, USA.

出版信息

Ital J Pediatr. 2024 Aug 6;50(1):141. doi: 10.1186/s13052-024-01698-7.

Abstract

BACKGROUND

Wilms tumor (WT) survival has been affected by the evolution in clinical and biological prognostic factors. Significant differences in survival rates indicate the need for further efforts to reduce these disparities. This study aims to evaluate the clinicopathological data impact on survival among patients after Wilm's diagnosis.

METHODS

The study utilized the SEERStat Database to identify Wilms tumor patients, applying SEERStat software version 8.3.9.2 for data extraction. Selection criteria involved specific codes based on the International Classification of Diseases for Oncology (ICDO-3), excluding cases with unknown SEER stage, incomplete survival data, unknown size, or lymph node status. Statistical analyses, including Kaplan-Meier estimates and Cox regression models, were conducted using R software version 3.5. Standardized mortality ratios (SMR) were computed with SEER*Stat software, and relative and conditional survival analyses were performed to evaluate long-term survival outcomes.

RESULTS

Of 2273 patients diagnosed with Wilms tumor, (1219 patients, 53.6% were females with an average age group of 3-8 years (50.2%). The overall mean survival after five years of diagnosis was 93.6% (2.6-94.7), and the overall mean survival rate was 92.5% (91.3-93.8) after ten years of diagnosis. Renal cancers were identified as the leading cause of death (77.3%), followed by nonrenal cancers (11%) and noncancer causes (11%). Additionally, robust relative survival rates of 98.10%, 92.80%, and 91.3% at one, five, and ten years, respectively, were observed, with corresponding five-year conditional survival rates indicating an increasing likelihood of survival with each additional year post-diagnosis. Univariate Cox regression identified significant prognostic factors: superior CSS for patients below 3 years (cHR 0.48) and poorer CSS for those older than 15 years (cHR 2.72), distant spread (cHR 10.24), regional spread (cHR 3.09), and unknown stage (cHR 4.97). In the multivariate model, age was not a significant predictor, but distant spread (aHR 9.22), regional spread (aHR 2.84), and unknown stage (aHR 4.98) were associated with worse CSS compared to localized tumors.

CONCLUSION

This study delving into WT survival dynamics reveals a multifaceted landscape influenced by clinicopathological variables. This comprehensive understanding emphasizes the imperative for ongoing research and personalized interventions to refine survival rates and address nuanced challenges across age, stage, and tumor spread in WT patients.

摘要

背景

威尔姆斯瘤(WT)的存活率受到临床和生物学预后因素演变的影响。生存率的显著差异表明需要进一步努力来减少这些差异。本研究旨在评估 WT 诊断后患者的临床病理数据对生存的影响。

方法

本研究利用 SEERStat 数据库来确定 WT 患者,使用 SEERStat 软件版本 8.3.9.2 进行数据提取。选择标准涉及基于国际肿瘤疾病分类(ICDO-3)的特定代码,排除 SEER 分期未知、生存数据不完整、大小或淋巴结状态未知的病例。使用 R 软件版本 3.5 进行统计分析,包括 Kaplan-Meier 估计和 Cox 回归模型。使用 SEER*Stat 软件计算标准化死亡率(SMR),并进行相对和条件生存分析,以评估长期生存结果。

结果

在 2273 名诊断为 WT 的患者中,(1219 名患者,53.6%为女性,平均年龄组为 3-8 岁(50.2%)。五年后诊断的总体平均生存率为 93.6%(2.6-94.7),十年后诊断的总体平均生存率为 92.5%(91.3-93.8)。肾癌是导致死亡的主要原因(77.3%),其次是非肾癌(11%)和非癌原因(11%)。此外,观察到相对生存率分别为 98.10%、92.80%和 91.3%,对应的五年条件生存率表明,随着诊断后时间的增加,生存的可能性增加。单因素 Cox 回归确定了显著的预后因素:年龄小于 3 岁的患者 CSS 更好(cHR 0.48),年龄大于 15 岁的患者 CSS 更差(cHR 2.72),远处扩散(cHR 10.24),区域扩散(cHR 3.09)和未知分期(cHR 4.97)。在多因素模型中,年龄不是一个显著的预测因素,但远处扩散(aHR 9.22)、区域扩散(aHR 2.84)和未知分期(aHR 4.98)与局部肿瘤相比,与较差的 CSS 相关。

结论

本研究深入探讨 WT 生存动态,揭示了受临床病理变量影响的多方面情况。这种全面的理解强调了需要进行持续的研究和个性化干预,以提高生存率,并解决 WT 患者在年龄、分期和肿瘤扩散方面的细微挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/28b1/11301993/16f84afaa341/13052_2024_1698_Fig1_HTML.jpg

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