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Title: | 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 |
Authors: | PASQUALINI, Claudia; FURTWAENGLER, Rhoikos; TINTEREN, Harm van; TEIXEIRA, Roberto A. P.; ACHA, Tomas; HOWELL, Lisa; VUJANIC, Gordan; GODZINSKI, Jan; MELCHIOR, Patrick; SMETS, Anne M.; COULOMB-L'HERMINE, Aurore; BRISSE, Herve; PRITCHARD-JONES, Kathy; BERGERON, Christophe; CAMARGO, Beatriz de; HEUVEL-EIBRINK, Marry M. Van den; GRAF, Norbert; VERSCHUUR, Arnauld C. |
Citation: | EUROPEAN JOURNAL OF CANCER, v.128, p.38-46, 2020 |
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: 1.6e33.3) and 4.9 months (range: 0.7 -28.4) for BT-WT and DA-WT, respectively (p Z 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. |
Appears in Collections: | Artigos e Materiais de Revistas Científicas - HC/ICr Artigos e Materiais de Revistas Científicas - LIM/36 Artigos e Materiais de Revistas Científicas - ODS/03 |
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