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Eur J Cancer. 2019 Jul;115:37-46. doi: 10.1016/j.ejca.2019.04.008. Epub 2019 May 10.

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

Author information

1
Department of Hygiene, Epidemiology and Medical Statistics, School of Medicine, National and Kapodistrian University of Athens, Greece; Oncology Department, "P & A Kyriakou" Children's Hospital, Athens, Greece.
2
Belarusian Research Center for Paediatric Oncology, Haematology and Immunology, Childhood Cancer Subregistry of Belarus, Minsk, Belarus.
3
Greater Poland Cancer Registry, Greater Poland Cancer Center, Poznan, Poland.
4
Cancer Registry of Slovenia, Institute of Oncology Ljubljana, Ljubljana, Slovenia.
5
Department of Hygiene, Epidemiology and Medical Statistics, School of Medicine, National and Kapodistrian University of Athens, Greece.
6
Oncology Department, "P & A Kyriakou" Children's Hospital, Athens, Greece.
7
Pediatric Hematology-Oncology Unit, First Department of Pediatrics, National and Kapodistrian University of Athens, "Agia Sofia" Children's Hospital, Athens, Greece.
8
Department of Pediatric Hematology-Oncology, "Agia Sofia" Children's Hospital, Athens, Greece.
9
Department of Pediatric Hematology and Oncology, Hippokration Hospital, Thessaloniki, Greece.
10
Hematology-Oncology Unit, 2nd Pediatric Department, Aristotle University of Thessaloniki, AHEPA Hospital, Thessaloniki, Greece.
11
Department of Pediatric Hematology-Oncology, University of Crete, Heraklion, Greece.
12
Pediatric Hematology-Oncology Department, "Mitera" Children's Hospital, Athens, Greece.
13
Pathology Laboratory, "Agia Sofia" Children's Hospital, Athens, Greece.
14
Pathology Laboratory, "P & A. Kyriakou" Children's Hospital, Athens, Greece.
15
Belarusian Research Center for Paediatric Oncology, Hematology and Immunology, Head of Oncology Department No1, Minsk, Belarus.
16
Patrycja Marciniak, University of Medical Sciences, Department of Pediatric Oncology Hematology and Transplantology, Poznan, Poland.
17
University Medical Center, Clinic of Pediatrics, University Children's Hospital, Ljubljana, Slovenia.
18
Department of Hygiene, Epidemiology and Medical Statistics, School of Medicine, National and Kapodistrian University of Athens, Greece; Clinical Epidemiology Unit, Department of Medicine, Karolinska Institute, Stockholm, Sweden. Electronic address: epetrid@med.uoa.gr.

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.

KEYWORDS:

Cancer registration; Event-free survival; Outcome predictors; Relapse; Wilms tumour

PMID:
31082691
DOI:
10.1016/j.ejca.2019.04.008

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