Sub-Department of Paediatric Surgery, College of Medicine, University of Nigeria, Enugu Campus, Enugu, Nigeria.
Department of Morbid Anatomy, College of Medicine, University of Nigeria, Enugu Campus, Enugu, Nigeria.
Pediatr Blood Cancer. 2019 Jan;66(1):e27416. doi: 10.1002/pbc.27416. Epub 2018 Aug 27.
BACKGROUND/OBJECTIVE: This study evaluates the outcome of Wilms tumor (WT) following introduction of multidisciplinary team management and patient treatment stratification by tumor histology in two referral centers in southeastern Nigeria.
We analyzed histologically confirmed WT cases managed from January 2008 to June 2017.
There were 45 patients, peak age incidence of 2 to 5 years who presented after mean symptom duration of 4.9 months (range, 1-12 months), with mean tumor weight of 1040 g (range, 350-4200 g). Overall, 14 (31.1%) had unfavorable histology of WT. A total of 22 (48.9%) patients received preoperative chemotherapy, 43 (95.6%) received postoperative chemotherapy based on stage of disease and histopathology, but none received adequate radiotherapy. Of these, 19 (44.2%) patients complied with chemotherapy regimen, 15 (33.3%) were lost to follow-up and 12 (26.7%) cases relapsed. With 30 cases available for evaluation and mean follow-up duration of 23 months (range, 6-80 months), the overall 5-year survival is 53.3% (16 cases). Survival in children who complied with postoperative chemotherapy was 73.7%, and abandonment-sensitive survival was 35.6%. Persisting challenges were late presentation, poor compliance to treatment, and lack of radiotherapy treatment.
Multidisciplinary team management and chemotherapy based on tumor histology might have resulted in slight improvement of outcome since our last report. However, to ensure survival that may approach global benchmarks, there is need for public health measures to improve time to diagnosis, and improvement of facilities and healthcare funding to ensure compliance to all phases of standard therapy.
背景/目的:本研究评估了在尼日利亚东南部的两个转诊中心引入多学科团队管理和基于肿瘤组织学的患者治疗分层后,威尔姆斯瘤(WT)的治疗结果。
我们分析了 2008 年 1 月至 2017 年 6 月间经组织学证实的 WT 病例。
共有 45 例患者,发病高峰年龄为 2 至 5 岁,平均症状持续时间为 4.9 个月(1-12 个月),平均肿瘤重量为 1040 克(350-4200 克)。总体而言,14 例(31.1%)WT 具有不良组织学特征。共有 22 例(48.9%)患者接受了术前化疗,43 例(95.6%)根据疾病分期和组织病理学接受了术后化疗,但均未接受足够的放疗。其中,19 例(44.2%)患者遵守了化疗方案,15 例(33.3%)失访,12 例(26.7%)病例复发。在 30 例可评估病例中,平均随访时间为 23 个月(6-80 个月),整体 5 年生存率为 53.3%(16 例)。遵守术后化疗的患儿存活率为 73.7%,放弃治疗敏感的存活率为 35.6%。持续存在的挑战是晚期就诊、治疗依从性差以及缺乏放疗治疗。
自我们上次报告以来,多学科团队管理和基于肿瘤组织学的化疗可能略微改善了治疗结果。然而,为了确保生存率接近全球基准,需要采取公共卫生措施来缩短诊断时间,并改善设施和医疗保健资金,以确保所有标准治疗阶段的治疗依从性。