Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years

被引:23
作者
Bergeron, C
Iliescu, C
Thiesse, P
Bouvier, R
Dijoud, F
Ranchere-Vince, D
Basset, T
Chappuis, JP
Buclon, M
Frappaz, D
Brunat-Mentigny, M
Philip, T
机构
[1] Ctr Leon Berard, Dept Pediat, F-69373 Lyon 08, France
[2] Hop Edouard Herriot, Hop Civils Lyon, Serv Anatomopathol & Chirurg Infantile, F-69437 Lyon 03, France
[3] Hop Debrousse, F-69322 Lyon 05, France
关键词
Wilms' tumour; nephroblastomas; nephroblastomatosis; chemotherapy; children;
D O I
10.1016/S0959-8049(00)00409-3
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
The presence of multifocal or diffuse nephrogenic rests (NRs) in one or both kidneys is termed nephroblastomatosis (Nbm). Nbm may be a predisposing factor for Wilms' tumour (WT). The aim of this retrospective study was to evaluate the impact of Nbm on the outcome of WT in children. We assessed the outcome of 81 children with Wilms tumours and practical implications of Nbm in the treatment and follow-up. All the pathology slides have been reviewed in 1997. 63 had WT without Nbm (group A) and 18 had WT associated with Nbm (group B). There was no statistical difference between the two groups according to the age at diagnosis and histology. Clinical abnormalities were more frequent in group B (33 versus 8%). There was no statistical difference between the percentage of stage IV in both groups, but bilaterality (stage V) was present only in the group B. Relapse was observed in 20/81 patients (25%). 11 (17%) in group A and 9 (50%) in group B. Mean delay of relapse was longer (25 months) in group B than in group A (10 months). For the whole population, with a median follow-up of 9 years, the event-free survival (EFS) and the overall survival (OS) probabilities were respectively 74%+/-10 and 83%+/-9 at 120 months. The difference in EFS between groups A (82+/-9%) and B (38%+/-29) was significant (P = 0.004). The discovery of Nbm in the non-tumoral part of the kidney with WT can be an adverse factor and in particular favours the subsequent development of a new Wilms tumour. It justifies separate follow-up guidelines. (C) 2001 Elsevier Science Ltd. All rights reserved.
引用
收藏
页码:385 / 391
页数:7
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