Complete necrosis induced by preoperative chemotherapy in Wilms tumor as an indicator of low risk: Report of the International Society of Paediatric Oncology (SIOP) Nephroblastoma Trial and Study 9

Author(s):  
Liliane Boccon-Gibod ◽  
Annie Rey ◽  
Bengt Sandstedt ◽  
Jan Delemarre ◽  
Dieter Harms ◽  
...  
2016 ◽  
Vol 34 (26) ◽  
pp. 3195-3203 ◽  
Author(s):  
Tasnim Chagtai ◽  
Christina Zill ◽  
Linda Dainese ◽  
Jenny Wegert ◽  
Suvi Savola ◽  
...  

Purpose Wilms tumor (WT) is the most common pediatric renal tumor. Treatment planning under International Society of Paediatric Oncology (SIOP) protocols is based on staging and histologic assessment of response to preoperative chemotherapy. Despite high overall survival (OS), many relapses occur in patients without specific risk factors, and many successfully treated patients are exposed to treatments with significant risks of late effects. To investigate whether molecular biomarkers could improve risk stratification, we assessed 1q status and other potential copy number biomarkers in a large WT series. Materials and Methods WT nephrectomy samples from 586 SIOP WT 2001 patients were analyzed using a multiplex ligation-dependent probe amplification (MLPA) assay that measured the copy number of 1q and other regions of interest. Results One hundred sixty-seven (28%) of 586 WTs had 1q gain. Five-year event-free survival (EFS) was 75.0% in patients with 1q gain (95% CI, 68.5% to 82.0%) and 88.2% in patients without gain (95% CI, 85.0% to 91.4%). OS was 88.4% with gain (95% CI, 83.5% to 93.6%) and 94.4% without gain (95% CI, 92.1% to 96.7%). In univariable analysis, 1q gain was associated with poorer EFS (P < .001; hazard ratio, 2.33) and OS (P = .01; hazard ratio, 2.16). The association of 1q gain with poorer EFS retained significance in multivariable analysis adjusted for 1p and 16q loss, sex, stage, age, and histologic risk group. Gain of 1q remained associated with poorer EFS in tumor subsets limited to either intermediate-risk localized disease or nonanaplastic localized disease. Other notable aberrations associated with poorer EFS included MYCN gain and TP53 loss. Conclusion Gain of 1q is a potentially valuable prognostic biomarker in WT, in addition to histologic response to preoperative chemotherapy and tumor stage.


2015 ◽  
Vol 14s4 ◽  
pp. CIN.S19340
Author(s):  
Sabine Müller ◽  
Ruslan David ◽  
Kostas Marias ◽  
Norbert Graf

The objective of this study is to assess standardized histograms of signal intensities of T2-weighted magnetic resonance image (MRI) modality before and after preoperative chemotherapy for nephroblastoma (Wilms’ tumor). All analyzed patients are enrolled in the International Society of Paediatric Oncology (SIOP) 2001/GPOH trial. 1 The question to be answered is whether the comparison of the histograms can add new knowledge by comparing them with the histology of the tumor after preoperative chemotherapy. Twenty-three unilateral nephroblastoma cases were analyzed. All patients were examined by MRI before and after preoperative chemotherapy treatment. T2 modalities of the MRIs were selected, and histogram changes were compared to histopathological data available after surgery. Of the 23 tumors, 22 decreased in volume following chemotherapy (median –57.99%; range 15.65 to –90.82%). The preliminary results suggest that standardized histograms of signal intensities of T2 MRI in nephroblastoma is not predicting histopathological diagnostic information and has no implications for the clinical assessment for further chemotherapy.


1988 ◽  
Vol 16 (1) ◽  
pp. 7-11 ◽  
Author(s):  
Guido Pastore ◽  
Modesto Carli ◽  
Jean Lemerle ◽  
Marie F. Tournade ◽  
Paul A. Voute ◽  
...  

1983 ◽  
Vol 1 (10) ◽  
pp. 604-609 ◽  
Author(s):  
J Lemerle ◽  
P A Voute ◽  
M F Tournade ◽  
C Rodary ◽  
J F Delemarre ◽  
...  

The results of a controlled clinical trial of preoperative radiotherapy compared to chemotherapy in patients with nephroblastoma are presented. Of 397 histologically proven cases of Wilms' tumor registered at 34 centers between January 1977 and July 1979, 164 were eligible for the trial and were randomized to receive preoperative radiotherapy and chemotherapy (group R, 76 patients) or preoperative chemotherapy (group C, 88 patients). The results were evaluated in terms of the number of surgical tumor ruptures and of local tumor extent at pathologic examination, reflecting the effectiveness of the preoperative treatment. Survival and recurrence-free survival in the two treatment groups were also taken into account. The stage distribution was comparable in the two groups, with 52% stage I tumors in group R, and 43% in group C. Significant changes in the pathologic pattern were more frequent in group R than in group C (53% versus 17%). From these data it is concluded that preoperative chemotherapy is as good as preoperative radiotherapy in terms of prevention of tumor rupture. In addition, it was shown that 43% of an unselected population of patients with Wilms' tumor could be treated without any radiotherapy when chemotherapy had been given preoperatively.


Author(s):  
Gordan M. Vujani? ◽  
Dieter Harms ◽  
Bengt Sandstedt ◽  
Angela Weirich ◽  
Jan de Kraker ◽  
...  

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