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Refining risk stratification for pretreated localised wilms tumours: the SIOP renal tumours srudy group experience [Abstract]

Pritchard-Jones, Kathy, van Tinteren, Harm, Sandstedt, Bengt, Vujanic, Gordan, Leuschner, Ivo, Boccon-Gibod, Liliane, de Camargo, Beatriz, Godzinski, Jan, Oldenberger, Foppe, Bergeron, Christophe, de Kraker, Jan and Graf, Norbert 2010. Refining risk stratification for pretreated localised wilms tumours: the SIOP renal tumours srudy group experience [Abstract]. Pediatric Blood and Cancer 55 (5) , p. 822. 10.1002/pbc.22779

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Purpose: The SIOP WT 2001 trial introduced a new ‘high risk’ entity: ‘blastemal type’ WT. However, the largest absolute number of relapses among localised tumours emanates from the ‘intermediate risk’ histology subgroup. We therefore investigated whether different thresholds for percentage necrosis/blastema might improve risk stratification based on histological response to pre-operative chemotherapy. Method: Data on 2,071 patients with localised unilateral WT treated with preoperative chemotherapy in the SIOP 2001 trial (to Sept 2009) were analysed. Martingale plots of excess risk of relapse versus overall% necrosis or%blastema in the viable residue were interrogated for thresholds at which risk altered. Event free survival was analysed by Kaplan-Meier methods and subgroups compared by log rank. Results: For the entire group, 2yr EFS was 88.2% (95%CI:86.6–89.8) and 5yr OS: 93.7% (95% CI:92.2–95.2). Histological risk group was a better discriminator of outcome than tumour stage (2yr EFS low risk:95.9%, intermediate risk:89.8% and high risk:76.9%, p<0.001; 2yr EFS stage I:91.0%, stage II:87.8% and stage III:83.2% p<0.001). Martingale plots showed no threshold effect for%necrosis but a reduced risk of relapse in those with <20% blastema in the viable tumour, with a small but steadily increasing risk of relapse with >50% blastema. For intermediate risk tumours, there was a significant decrease in EFS with increasing% blastema (comparing 0–10%, 10–90%, 90–100%). This persisted in the regressive subtype but was at the borderline for statistical significance in the mixed subtype (p¼0.05). The worst outcome group had 2 yr EFS of 79%. Conclusion: Survival of blastema after pre-operative chemotherapy in Wilms tumour is a better prognostic factor than% necrosis. Improved definition of chemoresistant blastema requires molecular characterisation of the disrupted biological pathways to improve risk stratification and inform discussions of new therapeutic approaches for these higher risk tumours.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Subjects: R Medicine > R Medicine (General)
R Medicine > RC Internal medicine > RC0254 Neoplasms. Tumors. Oncology (including Cancer)
R Medicine > RJ Pediatrics > RJ101 Child Health. Child health services
Additional Information: International Society of Paediatric Oncology SIOP XXXXII Congress Boston, United States October 21-24, 2010 SIOP Abstracts
Publisher: Wiley-Blackwell
ISSN: 1545-5009
Last Modified: 04 Jun 2017 03:15

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