scholarly journals Gain of 1q As a Prognostic Biomarker in Wilms Tumors (WTs) Treated With Preoperative Chemotherapy in the International Society of Paediatric Oncology (SIOP) WT 2001 Trial: A SIOP Renal Tumours Biology Consortium Study

2016 ◽  
Vol 34 (26) ◽  
pp. 3195-3203 ◽  
Author(s):  
Tasnim Chagtai ◽  
Christina Zill ◽  
Linda Dainese ◽  
Jenny Wegert ◽  
Suvi Savola ◽  
...  
Keyword(s):  
Preoperative Chemotherapy ◽  
International Society ◽  
Copy Number ◽  
Prognostic Biomarker ◽  
Wilms Tumor ◽  
Multivariable Analysis ◽  
Paediatric Oncology ◽  
Hazard Ratio ◽  
Localized Disease ◽  
1Q Gain

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.

scholarly journals Association of Chromosome 1q Gain With Inferior Survival in Favorable-Histology Wilms Tumor: A Report From the Children’s Oncology Group

2016 ◽  
Vol 34 (26) ◽  
pp. 3189-3194 ◽  
Author(s):  
Eric J. Gratias ◽  
Jeffrey S. Dome ◽  
Lawrence J. Jennings ◽  
Yueh-Yun Chi ◽  
Jing Tian ◽  
...  
Keyword(s):  
Copy Number ◽  
Wilms Tumor ◽  
Multivariable Analysis ◽  
Stage Iv ◽  
Stage I ◽  
Disease Stage ◽  
Copy Number Loss ◽  
Entire Cohort ◽  
Favorable Histology ◽  
1Q Gain

Purpose The goal of this study was to analyze the association of copy number gain of 1q in favorable-histology Wilms tumors (FHWTs) with event-free survival (EFS) and overall survival (OS) within each tumor stage and with 1p and 16q copy number loss and/or loss of heterozygosity. Methods Unilateral FHWTs from 1,114 patients enrolled in National Wilms Tumor Study-5 that were informative for 1p and 16q microsatellite markers (previously determined) and informative for 1q gain, 1p loss, and 16q loss using multiplex ligation-dependent probe amplification were analyzed. Results Eight-year EFS was 86% (95% CI, 84% to 88%) for the entire cohort. Of 1,114 patients, 317 tumors (28%) displayed 1q gain. Eight-year EFS was 77% for those with 1q gain and 90% for those lacking 1q gain (P < .001). Eight-year OS was 88% for those with 1q gain and 96% for those lacking 1q gain (P < .001). Within each disease stage, 1q gain was associated with inferior EFS (stage I, 85% v 95%; P = .0052; stage II, 81% v 87%; P = .0775; stage III, 79% v 89%; P = .01; stage IV, 64% v 91%; P = .001). OS was significantly inferior in patients with stage I (P < .0015) and stage IV disease (P = .011). With multivariable analysis, 1q gain was associated with an increased relative risk of relapse of 2.4 (P < .001), whereas 1p loss was not, despite significance on univariable analysis. Conclusion Gain of 1q is associated with inferior survival in unilateral FHWTs and may be used to guide risk stratification in future studies.

scholarly journals The Standardized Histogram Shift of T2 Magnetic Resonance Image (MRI) Signal Intensities of Nephroblastoma Does Not Predict Histopathological Diagnostic Information

2015 ◽  
Vol 14s4 ◽  
pp. CIN.S19340
Author(s):  
Sabine Müller ◽  
Ruslan David ◽  
Kostas Marias ◽  
Norbert Graf
Keyword(s):  
Magnetic Resonance ◽  
Preoperative Chemotherapy ◽  
Magnetic Resonance Image ◽  
Wilms Tumor ◽  
Paediatric Oncology ◽  
Diagnostic Information ◽  
Chemotherapy Treatment ◽  
Before And After ◽  
T2 Mri ◽  
Signal Intensities

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.

Epidemiological features of Wilms' tumor: Results of studies by the international society of paediatric oncology (SIOP)

1988 ◽  
Vol 16 (1) ◽  
pp. 7-11 ◽  
Author(s):  
Guido Pastore ◽  
Modesto Carli ◽  
Jean Lemerle ◽  
Marie F. Tournade ◽  
Paul A. Voute ◽  
...  
Keyword(s):  
International Society ◽  
Wilms Tumor ◽  
Paediatric Oncology ◽  
Epidemiological Features

scholarly journals Treatment of Wilms Tumor in Sub-Saharan Africa: Results of the Second French African Pediatric Oncology Group Study

2019 ◽  
pp. 1-8 ◽  
Author(s):  
Atteby Jean-Jacques Yao ◽  
Claude Moreira ◽  
Fousseyni Traoré ◽  
Sonia Kaboret ◽  
Angele Pondy ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Stage Iv ◽  
Paediatric Oncology ◽  
Sub Saharan Africa ◽  
Stage Iii ◽  
Postoperative Treatment ◽  
Pediatric Oncology Group ◽  
Localized Disease ◽  
Sub Saharan ◽  
Chemotherapy Patients

PURPOSE Multidisciplinary management of Wilms tumor has been defined through multicenter prospective studies and an average expected patient cure rate of 90%. In sub-Saharan Africa, such studies are uncommon. After the encouraging results of the first Groupe Franco-Africain d'Oncologie Pédiatrique (GFAOP) study, we report the results of the GFAOP-NEPHRO-02 study using an adaptation of the International Society of Paediatric Oncology 2001 protocol. PATIENTS AND METHODS From April 1, 2005, to March 31, 2011, seven African units participated in a nonrandomized prospective study. All patients who were referred with a clinical and radiologic diagnosis of renal tumor were screened. Those older than age 6 months and younger than 18 years with a unilateral tumor previously untreated were pre-included and received preoperative chemotherapy. Patients with unfavorable histology or with a tumor other than Wilms, or with a nonresponding stage IV tumor were excluded secondarily. RESULTS Three hundred thirteen patients were initially screened. Two hundred fifty-seven patients were pre-included and 169 with histologic confirmation of intermediate-risk nephroblastoma were registered in the study and administered postoperative treatment. Thirty-one percent of patients were classified as stage I, 38% stage II, 24% stage III, and 7% stage IV. Radiotherapy was not available for any stage III patients. Three-year overall survival rate was 72% for all study patients and 73% for those with localized disease. CONCLUSION It was possible to conduct sub-Saharan African multicenter therapeutic studies within the framework of GFAOP. Survival results were satisfactory. Improvements in procedure, data collection, and outcome are expected in a new study. Radiotherapy is needed to reduce the relapse rate in patients with stage III disease.

Effectiveness of preoperative chemotherapy in Wilms' tumor: results of an International Society of Paediatric Oncology (SIOP) clinical trial.

1983 ◽  
Vol 1 (10) ◽  
pp. 604-609 ◽  
Author(s):  
J Lemerle ◽  
P A Voute ◽  
M F Tournade ◽  
C Rodary ◽  
J F Delemarre ◽  
...  
Keyword(s):  
Clinical Trial ◽  
Preoperative Chemotherapy ◽  
Preoperative Radiotherapy ◽  
Wilms Tumor ◽  
Paediatric Oncology ◽  
Controlled Clinical Trial ◽  
Preoperative Treatment ◽  
Treatment Groups ◽  
Chemotherapy Group ◽  
Unselected Population

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.

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