Wilms tumour event-free and overall survival in Southern and Eastern Europe: Pooled analyses of clinical data from four childhood cancer registries (1999–2017). (July 2019)

• Record Type: Journal Article
• Authors: Doganis, Dimitrios; Zborovskaya, Anna; Trojanowski, Maciej; Zagar, Tina; Bouka, Panagiota; Baka, Margaret; Moschovi, Maria; Polychronopoulou, Sofia; Papakonstantinou, Eugenia; Tragiannidis, Athanasios; Stiakaki, Eftichia; Dana, Helen; Stefanaki, Kalliopi; Strantzia, Katerina; Kochubinsky, Dmitry; Marciniak, Patrycja; Avcin, Simona; Antoniadi, Kondilia; Dessypris, Nick; Petridou, Eleni Th.
• Is Part Of: European journal of cancer. Volume 115(2019)
• Page Start: 37
• Page End: 46
• Publication Date: 2019-07
• Subjects: Wilms tumour -- Event-free survival -- Relapse -- Cancer registration -- Outcome predictors; Cancer -- Periodicals; Neoplasms -- Periodicals; Cancer -- Périodiques; Cancer; Tumors; Electronic journals; Periodicals; Electronic journals; 616.994
• DOI: 10.1016/j.ejca.2019.04.008
• Languages: English
• ISSNs: 0959-8049

Abstract

Abstract: Background: Wilms tumour (WT) management represents a success story in pediatric oncology. We aimed to assess, for the first time, the event-free survival (EFS) vs. overall survival (OS) in Southern and Eastern Europe (SEE) using harmonised clinical data collected by childhood cancer registries and to identify respective prognostic factors. Methods: From 1999 to 2017, data for incident WT cases aged 0–14 years from 3 nationwide (Greece, Belarus and Slovenia) and one regional (Greater Poland) SEE registries were collected following common coding. Kaplan–Meier curves were constructed, and EFS vs. OS values were derived from Cox proportional hazard models by study variables. Results: A total of 338 WT cases (45.6% males; median age, 3.19 years; age<5 years, 75%) were included in the analyses. Bilateral were 21 tumours (6.2%). Among the 317 unilateral cases, the majority (93.7%) received International Society of Pediatric Oncology–based protocols; EFS5-year was 85.1%, and OS5-year 91.1%; both outcomes were significantly worse in stage IV patients or in those with high-risk/unfavourable histology. Relapse rate among high-risk/unfavourable histology cases was 2.3 times higher than among low-intermediate risk/favourable histology cases, with respective death rate 5.6 times higher. Both relapse and death rates increased significantly in patients with advanced anatomical stage and high-risk/unfavourable histology. Finally, significantly worse was the outcome in bilateral tumours (OS5-year : 76.3%) vs. unilateral non-metastatic tumours (OS5-year : 94.7%). Conclusions: Our results delineate the potential of high-quality childhood cancer registration entailing clinical data to assess predictors of WT outcome over and beyond those derived from enrolment into clinical trials. Specifically, outcomes among children with WT residing in the four participating SEE countries were comparable with those reported by major cooperative international groups, albeit somehow inferior. Despite the excellent overall prognosis, however, subgroups of patients with advanced or bilateral disease and/or high-risk histology still suffer poor outcomes. Highlights: Event-free survival (EFS) and overall survival (OS) in South-East Europe are comparable with those of major cooperative international groups. Outcomes for patients with advanced disease, high-risk histology and bilateral tumours need to be optimised. Better risk stratification and enhanced clinical management may be needed to improve outcomes. Quality childhood cancer registration entailing harmonised clinical data is crucial in assessing Wilms tumour outcomes.