Abstract
Blastemal-type Wilms tumour (BT-WT) has been identified as a high risk histological
subgroup in WT assessed after pre-nephrectomy chemotherapy in trials of the International
Society of Paediatric Oncology (SIOP) Renal Tumour Study Group. Therefore, in SIOPWT2001,
post-operative chemotherapy for BT-WT was intensified aiming to improve survival.
Survival analysis of all unilateral BT-WT patients (SIOPWT2001) (n = 238), was compared with historical BT-WT controls (SIOP93-01) (n = 113). 351/4061 (8.6%) unilateral non-metastatic BT-WT patients (SIOP93-01/SIOPWT2001)
were studied. Median age at diagnosis was 43 months (Inter Quartile Range (IQR) 24–68 months), stages: I (n = 140, 40%), II (n = 106, 30%), III (n = 105, 30%). BT-WTs were higher staged, showed greater volume decrease after pre-operative
chemotherapy and were diagnosed at an older median age compared to other WT patients.
Patient characteristics did not differ substantially between SIOP93-01 and SIOPWT2001.
Univariate analysis showed a 5-year event-free survival (EFS) of 80% (95% confidence
interval (CI): 75–86%) (SIOPWT2001) compared to 67% in SIOP93-01 (95% CI: 59–76%;
p = 0.006) and overall survival (OS) of 88% (95% CI: 83–93%) (SIOPWT2001) compared to
84% (95% CI: 77–91%; p = 0.4) in SIOP93-01. 95% of relapses were distant metastases (SIOP93-01/SIOPWT2001).
Treatment protocol, age at diagnosis, tumour stage (III versus I/II) and volume (at
surgery), were prognostic variables for EFS (uni- and multivariate Cox regression
analysis). Independent prognosticators for OS were age at diagnosis, tumour stage
and volume (at surgery). The most significant survival benefit of intensified treatment,
was observed in Stage I (EFS 96% in SIOPWT2001 (OS 100%), 71% in SIOP93-01 (OS 90%)).
BT-WT derived benefits from more intensive chemotherapy as reflected by a reduction
in relapse risk. However, the benefit of the more intensive chemotherapy to improve
OS was only observed in stage I BT-WTs, by adding doxorubicin.
Keywords
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Article info
Publication history
Published online: January 13, 2015
Accepted:
December 14,
2014
Received in revised form:
November 20,
2014
Received:
August 27,
2014
Identification
Copyright
© 2014 Elsevier Ltd. Published by Elsevier Inc. All rights reserved.
