RT Journal Article
T1 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.
A1 Pasqualini, Claudia
A1 Furtwängler, Rhoikos
A1 van Tinteren, Harm
A1 Teixeira, Roberto A P
A1 Acha, Tomas
A1 Howell, Lisa
A1 Vujanic, Gordan
A1 Godzinski, Jan
A1 Melchior, Patrick
A1 Smets, Anne M
A1 Coulomb-L'Hermine, Aurore
A1 Brisse, Hervé
A1 Pritchard-Jones, Kathy
A1 Bergeron, Christophe
A1 de Camargo, Beatriz
A1 van den Heuvel-Eibrink, Marry M
A1 Graf, Norbert
A1 Verschuur, Arnauld C
K1 Anaplasia
K1 Blastema
K1 Cancer
K1 Child
K1 TP53
K1 Wilms
AB High-risk (HR) metastatic (stage IV) Wilms tumours (WTs) have a particular poor outcome. 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. 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. These results call for new treatment approaches for patients with HR stage IV WT.
YR 2020
FD 2020-03-05
LK http://hdl.handle.net/10668/15181
UL http://hdl.handle.net/10668/15181
LA en
DS RISalud
RD Apr 6, 2025