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Eur J Cancer. 2020 Feb 7;128:38-46. doi: 10.1016/j.ejca.2020.01.001. [Epub ahead of print]

Outcome of patients with stage IV high-risk Wilms tumour treated according to the SIOP2001 protocol: A report of the SIOP Renal Tumour Study Group.

Author information

1
Children and Adolescents Oncology Department, Gustave Roussy, Villejuif, France. Electronic address: claudia.pasqualini@gustaveroussy.fr.
2
Department of Pediatric Haematology/Oncology, Saarland University Hospital, Homburg, Germany. Electronic address: rhoikos.furtwaengler@uks.eu.
3
Biometrics Department, Netherlands Cancer Institute, Amsterdam, the Netherlands. Electronic address: h.v.tinteren@nki.nl.
4
Instituto Do Tratamento Do Câncer Infantil, Department of Pediatrics, São Paulo University, São Paulo, Brazil. Electronic address: rapteixeira@uol.com.br.
5
Hospital Materno-Infantil "Carlos Haya", Department of Pediatrics, Malaga, Spain. Electronic address: tomas.acha.sspa@juntadeandalucia.es.
6
Department of Oncology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom. Electronic address: lisa.howell@alderhey.nhs.uk.
7
Department of Pathology, Sidra Medicine, Doha, Qatar. Electronic address: gvujanic@sidra.org.
8
Department of Pediatric Surgery, Marciniak Hospital Wroclaw, Wroclaw, Poland; Department of Pediatric Traumatology and Emergency Medicine, Medical University, Wroclaw, Poland. Electronic address: jgodzin@wp.pl.
9
Department of Radiation Oncology, Saarland University Hospital, Homburg, Germany. Electronic address: patrick.melchior@uks.eu.
10
Department of Radiology and Nuclear Medicine, Academic Medical Center, Amsterdam, the Netherlands. Electronic address: a.m.smets@amc.uva.nl.
11
Department of Pathology, Hospital D'Enfants Armand Trousseau, Paris, France. Electronic address: aurore.coulomb@aphp.fr.
12
Department of Radiology, Curie Institut, Paris, France. Electronic address: herve.brisse@curie.fr.
13
Great Ormond Street Institute of Child Health, University College London, London, UK. Electronic address: k.pritchard-jones@ucl.ac.uk.
14
Pediatric Onco-Haematology Department, Centre Leon Berard, Lyon, France. Electronic address: christophe.bergeron@ihope.fr.
15
Instituto Nacional Do Cancer, Pediatric Onco-Haematology, Rio de Janeiro, Brazil. Electronic address: bdecamar@terra.com.br.
16
Oncology, Princess Maxima Centre for Pediatric Oncology, Utrecht, the Netherlands; Dutch Childhood Oncology Group, The Hague, the Netherlands. Electronic address: m.m.vandenheuvel-eibrink@prinsesmaximacentrum.nl.
17
Department of Pediatric Haematology/Oncology, Saarland University Hospital, Homburg, Germany. Electronic address: graf@uks.eu.
18
Pediatric Onco-Haematology Department, Hopital de La Timone, AP-HM, Marseille, France. Electronic address: arnauld.verschuur@ap-hm.fr.

Abstract

INTRODUCTION:

High-risk (HR) metastatic (stage IV) Wilms tumours (WTs) have a particular poor outcome.

METHODS:

Here, we report the results of HR (diffuse anaplastic [DA] or blastemal type [BT]) stage IV WT treated patients according to the HR arm in the SIOP2001 prospective study.

RESULTS:

From January 2002 to August 2014, 3559 patients with WT were included in the SIOP2001 trial. Among the 525 patients (15%) with metastatic WT, 74 (14%) had stage IV HR-WT. The median age at diagnosis was 5.5 years (range: 1.4-18.3). Thirty-four patients (47%) had BT-WT and 40 (53%) had DA-WT. Five-year event-free survival rates were 44 ± 17% and 28 ± 15% for BT-WT and DA-WT, respectively (p = 0.09). Five-year overall survival rates were 53 ± 17% and 29 ± 16% for BT-WT and DA-WT, respectively (p = 0.03). Metastatic complete response after preoperative treatment was significantly associated with outcome in univariate and multivariate analyses (hazards ratio = 0.3; p = 0.01). Postoperative radiotherapy of metastatic sites might also be beneficial. Forty-three of 74 patients experienced a relapse or progression predominantly in the lungs (80%). The median time to relapse/progression after diagnosis was 7.3 months (range: 1.6-33.3) and 4.9 months (range: 0.7-28.4) for BT-WT and DA-WT, respectively (p = 0.67). This is the first prospective evidence of inferior survival of stage IV BT-WT as compared with historical intermediate-risk WT. Survival of patients with stage IV DA-WT has not improved compared to the previous SIOP93-01 study.

CONCLUSION:

These results call for new treatment approaches for patients with HR stage IV WT.

KEYWORDS:

Anaplasia; Blastema; Cancer; Child; TP53; Wilms

PMID:
32109849
DOI:
10.1016/j.ejca.2020.01.001

Conflict of interest statement

Conflict of interest statement The authors declare no conflict of interest.

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