Journal
EUROPEAN JOURNAL OF CANCER
Volume 128, Issue -, Pages 38-46Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.ejca.2020.01.001
Keywords
Wilms; Anaplasia; Blastema; Cancer; Child; TP53
Categories
Funding
- German CancerAid (Deutsche Krebshilfe) [50-2709-GR2]
- Association Leon Berard Enfant Cancereux
- Enfant et Sante(Albec)
- DaDa Society (Nieuwerkerk a/d Ijssel, The Netherlands)
- Paediatric Oncology Centre Society for Research (KOCR, Rotterdam, the Netherlands)
- Grupo Cooperativo Brasileiro para o Tratamento do tumour de Wilms (GCBTTW)
- Spanish Association Against Cancer (AECC)
- Cancer Research UK [C1010/A2889, C1188/A8687]
- National Cancer Research Network
- Societe Francaise des Cancers de l'Enfant (SFCE)
- Gesellschaft fur Paediatrische Onkologie und Haematologie (GPOH)
- Children's Cancer and Leukemia Group (CCLG)
- Spanish Society of Paediatric Haematology and Oncology (SEHOP)
- Sociedade Brasileira de Oncologia Pediatrica (SOBOPE)
- Stichting Kinderoncologie Nederland (SKION)
Ask authors/readers for more resources
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. (C) 2020 Elsevier Ltd. All rights reserved.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available