Rahiman Emine A, Trehan Amita, Jain Richa, Menon Prema, Kakkar Nandita, Srinivasan Radhika, Sodhi Kushaljit Singh, Saxena Akshay Kumar, Kapoor Rakesh, Bansal Deepak
Pediatric Hematology-Oncology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Pediatrics, All India Institute of Medical Sciences, Bibinagar, Hyderabad, Telangana, India.
Pediatr Blood Cancer. 2022 Nov;69(11):e29880. doi: 10.1002/pbc.29880. Epub 2022 Jul 16.
Distinct prognostic factors for Wilms tumor (WT) in low- and middle-income countries need identification.
Retrospective study of patients with WT managed by the International Society of Pediatric Oncology (SIOP) approach for over 11 years (2005-2016) at a single center in Chandigarh, India.
The study included 200 patients (median age: 33.5 months). The tumor stage (SIOP) distribution included stage I (30%), II (36%), III (14%), IV (17%), and V (3%). The histology-risk groups were low (8%), intermediate (84%), and high risk (9%). At diagnosis, 68 out of 190 (36%) patients were underweight. The median tumor volume at diagnosis was 481 ml (interquartile ratio [IQR]: 306.9, 686.8, n = 146). Following neoadjuvant chemotherapy, it reduced to 110 ml (IQR: 151.2, 222, n = 77). Treatment was abandoned in 20.5% of the patients. Treatment-related mortality occurred in 13 of 179 (7.2%) patients. Relapse occurred in 26 of 158 (16.5%) patients. The 3-year overall survival (OS) and event-free survival (EFS) of patients who completed therapy were 78.3 and 72%, respectively. The stage (p = .013) and histology (p = .023) influenced OS. A lower OS in stage II (75.4%) versus stage III disease (83.7%) suggested understaging. Patients with a higher tumor volume at diagnosis (p = .005; odds ratio [OR]: 0.99; 95% confidence interval [CI]: 0.99-1.00) or a lower weight-for-age z-score (p = .002; OR: 1.68; 95% CI: 1.21-2.33) had an increased risk of death or relapse.
The 3-year OS and EFS of children who completed therapy were 78.3 and 72%, respectively. A higher tumor volume and lower weight-for-age z-score at diagnosis were identified as distinct adverse prognostic factors. A likely suboptimal lymph node assessment (intraoperative and histopathology) contributed to the understaging of stage III to II disease and reduced survival.
需要确定低收入和中等收入国家中威尔姆斯瘤（WT）的不同预后因素。
对在印度昌迪加尔的一个中心采用国际小儿肿瘤学会（SIOP）方法管理超过11年（2005 - 2016年）的WT患者进行回顾性研究。
该研究纳入200例患者（中位年龄：33.5个月）。肿瘤分期（SIOP）分布为I期（30%）、II期（36%）、III期（14%）、IV期（17%）和V期（3%）。组织学风险组为低风险（8%）、中风险（84%）和高风险（9%）。诊断时，190例患者中有68例（36%）体重过轻。诊断时肿瘤体积中位数为481毫升（四分位间距[IQR]：306.9，686.8，n = 146）。新辅助化疗后，肿瘤体积降至110毫升（IQR：151.2，222，n = 77）。20.5%的患者放弃治疗。179例患者中有13例（7.2%）发生治疗相关死亡。158例患者中有26例（16.5%）复发。完成治疗的患者3年总生存率（OS）和无事件生存率（EFS）分别为78.3%和72%。分期（p = 0.013）和组织学（p = 0.023）影响总生存率。II期疾病（75.4%）的总生存率低于III期疾病（83.7%），提示分期不足。诊断时肿瘤体积较大（p = 0.005；比值比[OR]：0.99；95%置信区间[CI]：0.99 - 1.00）或年龄别体重z评分较低（p = 0.002；OR：1.68；95% CI：1.21 - 2.33）的患者死亡或复发风险增加。
完成治疗的儿童3年总生存率和无事件生存率分别为78.3%和72%。诊断时较高的肿瘤体积和较低的年龄别体重z评分被确定为明显的不良预后因素。可能是次优的淋巴结评估（术中及组织病理学）导致III期疾病被误分期为II期并降低了生存率。
