The Experts below are selected from a list of 276 Experts worldwide ranked by ideXlab platform
Norman E. Breslow - One of the best experts on this subject based on the ideXlab platform.
-
Risk Factors for End Stage Renal Disease in Non-WT1-Syndromic Wilms Tumor
The Journal of Urology, 2011Co-Authors: Jane M. Lange, Susan M. Peterson, Janice R. Takashima, Yevgeny A. Grigoriev, Daniel M Green, Michael L Ritchey, Robert C Shamberger, J. Bruce Beckwith, Elizabeth J. Perlman, Norman E. BreslowAbstract:Purpose: We assessed risk factors for end stage renal disease in patients with Wilms Tumor without known WT1 related syndromes. We hypothesized that patients with characteristics suggestive of a WT1 etiology (early onset, stromal predominant histology, intralobar nephrogenic rests) would have a higher risk of end stage renal disease due to chronic renal failure. We predicted a high risk of end stage renal disease due to progressive bilateral Wilms Tumor in patients with metachronous bilateral disease.Materials and Methods: End stage renal disease was ascertained in 100 of 7,950 nonsyndromic patients enrolled in a National Wilms Tumor Study during 1969 to 2002. Risk factors were evaluated with cumulative incidence curves and proportional hazard regressions.Results: The cumulative incidence of end stage renal disease due to chronic renal failure 20 years after Wilms Tumor diagnosis was 0.7%. For end stage renal disease due to progressive bilateral Wilms Tumor the incidence was 4.0% at 3 years after diagnosi...
-
treatment of anaplastic histology Wilms Tumor results from the fifth national Wilms Tumor study
Journal of Clinical Oncology, 2006Co-Authors: Jeffrey S Dome, Norman E. Breslow, Paul E Grundy, Michael L Ritchey, Bruce J Beckwith, Elizabeth J. Perlman, Cecilia A Cotton, John A Kalapurakal, Marcio H Malogolowkin, Robert C ShambergerAbstract:Purpose An objective of the fifth National Wilms' Tumor Study (NWTS-5) was to evaluate the efficacy of treatment regimens for anaplastic histology Wilms' Tumor (AH). Patients and Methods Prospective single-arm studies were conducted. Patients with stage I AH were treated with vincristine and dactinomycin for 18 weeks. Patients with stages II to IV diffuse AH were treated with vincristine, doxorubicin, cyclophosphamide, and etoposide for 24 weeks plus flank/abdominal radiation. Results A total of 2,596 patients with Wilms' Tumor were enrolled onto NWTS-5, of whom 281 (10.8%) had AH. Four-year event-free survival (EFS) and overall survival (OS) estimates for assessable patients with stage I AH (n = 29) were 69.5% (95% CI, 46.9 to 84.0) and 82.6% (95% CI, 63.1 to 92.4). In comparison, 4-year EFS and OS estimates for patients with stage I favorable histology (FH; n = 473) were 92.4% (95% CI, 89.5 to 94.5) and 98.3% (95% CI, 96.4 to 99.2). Four-year EFS estimates for patients who underwent immediate nephrectom...
-
end stage renal disease in patients with Wilms Tumor results from the national Wilms Tumor study group and the united states renal data system
The Journal of Urology, 2005Co-Authors: Norman E. Breslow, Susan M. Peterson, Michael L Ritchey, Allan J Collins, Yevgeny Grigoriev, Daniel M GreenAbstract:ABSTRACTPurpose: We sought to assess accurately the full spectrum of end stage renal disease (ESRD) in Wilms Tumor survivors by combining the unique resources of the National Wilms Tumor Study Grou...
-
intravascular extension of Wilms Tumor
Annals of Surgery, 2001Co-Authors: Robert C Shamberger, Norman E. Breslow, Michael L Ritchey, Gerald M Haase, Tracy L Bergemann, Teri Loecheltyoshioka, Daniel M GreenAbstract:ObjectiveTo define the incidence and manifestations of and optimal therapy for children with intravascular extension of Wilms Tumor.MethodsChildren on a collaborative study of Wilms Tumor who had intravascular extension into the inferior vena cava (IVC) or atrium were identified. Surgical checklists
-
surgical complications after primary nephrectomy for Wilms Tumor report from the national Wilms Tumor study group
Journal of The American College of Surgeons, 2001Co-Authors: Michael L Ritchey, Robert C Shamberger, Gerald M Haase, Tracy L Bergemann, Jeffrey R Horwitz, Norman E. BreslowAbstract:Abstract BACKGROUND: Surgical complications are a recognized morbidity of the treatment of patients with Wilms Tumor. This study examines the incidence of surgical complications in the most recently completed study from the National Wilms' Tumor Study Group (NWTSG). STUDY DESIGN: The fourth National Wilms' Tumor Study (NWTS-4) enrolled 3,335 patients from August 1986 to August 1994. A random sample of 534 patients was selected from 2,290 eligible patients randomized to treatment regimens or enrolled in the followed category and treated according to NWTSG protocol. The patient records received at the NWTSG Data and Statistical Center were analyzed for surgical complications (intraoperative and postoperative). RESULTS: Sixty-eight patients (12.7%) experienced 76 complications. Intestinal obstruction was the most common complication (5.1% of patients), followed by extensive hemorrhage (1.9%), wound infection (1.9%), and vascular injury (1.5%). The incidence of surgical complications in NWTS-4 was significantly lower than NWTS-3 (12.7% versus 19.8%, p CONCLUSIONS: The incidence of surgical complications in NWTSG patients undergoing primary nephrectomy has significantly decreased over the past decade. But surgical morbidity should not be overlooked. It is important that surgeons treating young children with solid Tumors are aware of their role and the potential risks encountered in removal of the primary Tumor. This study found that surgical specialists who primarily treat children can perform these operations with lower surgical morbidity.
Daniel M Green - One of the best experts on this subject based on the ideXlab platform.
-
lymph node involvement in Wilms Tumor results from national Wilms Tumor studies 4 and 5
Journal of Pediatric Surgery, 2012Co-Authors: Kathleen Kieran, Daniel M Green, Michael L Ritchey, Robert C Shamberger, Elizabeth J. Perlman, Peter F Ehrlich, Jeffrey S Dome, James R Anderson, Andrew M. DavidoffAbstract:Purpose The aim of the study was to determine the prognostic impact of lymph node (LN) involvement and sampling in patients with Wilms Tumor (WT) and the minimum number of LNs needed for accurate staging.
-
Risk Factors for End Stage Renal Disease in Non-WT1-Syndromic Wilms Tumor
The Journal of Urology, 2011Co-Authors: Jane M. Lange, Susan M. Peterson, Janice R. Takashima, Yevgeny A. Grigoriev, Daniel M Green, Michael L Ritchey, Robert C Shamberger, J. Bruce Beckwith, Elizabeth J. Perlman, Norman E. BreslowAbstract:Purpose: We assessed risk factors for end stage renal disease in patients with Wilms Tumor without known WT1 related syndromes. We hypothesized that patients with characteristics suggestive of a WT1 etiology (early onset, stromal predominant histology, intralobar nephrogenic rests) would have a higher risk of end stage renal disease due to chronic renal failure. We predicted a high risk of end stage renal disease due to progressive bilateral Wilms Tumor in patients with metachronous bilateral disease.Materials and Methods: End stage renal disease was ascertained in 100 of 7,950 nonsyndromic patients enrolled in a National Wilms Tumor Study during 1969 to 2002. Risk factors were evaluated with cumulative incidence curves and proportional hazard regressions.Results: The cumulative incidence of end stage renal disease due to chronic renal failure 20 years after Wilms Tumor diagnosis was 0.7%. For end stage renal disease due to progressive bilateral Wilms Tumor the incidence was 4.0% at 3 years after diagnosi...
-
bilateral Wilms Tumor with anaplasia lessons from the national Wilms Tumor study
Journal of Pediatric Surgery, 2006Co-Authors: Thomas E Hamilton, Daniel M Green, Pedram Argani, Paul E Grundy, Michael L Ritchey, Elizabeth J. Perlman, Robert C ShambergerAbstract:Abstract Purpose The purpose of this study was to evaluate whether initial diagnostic technique influenced the ability to identify anaplastic histology, to determine the time interval to diagnosis of anaplasia, and to delineate the incidence of discordant pathology in bilateral Wilms' Tumor. We hypothesized that delay in diagnosis of anaplasia could affect time to appropriate surgery and intensive multimodality therapy. Methods One hundred eight-nine children were enrolled in the fourth National Wilms' Tumor Study with synchronous bilateral Tumors, 27 of whom were eventually shown to have anaplastic histology. Initial diagnostic technique, time interval to diagnosis of anaplasia, and the incidence of discordant pathology were determined. Results Anaplasia was identified in 0 of 7 Tumors by core needle biopsy, 3 of 9 Tumors by open wedge biopsy, and in 7 of 9 cases by partial or complete nephrectomy. The mean duration of first chemotherapy regimen (DD or EE) was 20, 39, and 36 weeks, respectively, before anaplasia was identified at second surgery. Discordant pathology between bilateral Tumors was identified on final tissue diagnosis in 20 patients. Only 4 patients had anaplastic Tumors in both kidneys. Conclusions Core needle biopsy did not identify anaplasia in 7 of 7 children. Open biopsy or partial/complete nephrectomy identified anaplasia at initial diagnostic procedure in 10 of 18 children. Twenty of 24 patients at final tissue diagnosis had discordant pathology between the 2 kidneys. Earlier interval incisional biopsy or resection may identify anaplastic histology and limit the duration of chemotherapy targeted to favorable histology for children with bilateral Wilms' Tumor and anaplasia.
-
end stage renal disease in patients with Wilms Tumor results from the national Wilms Tumor study group and the united states renal data system
The Journal of Urology, 2005Co-Authors: Norman E. Breslow, Susan M. Peterson, Michael L Ritchey, Allan J Collins, Yevgeny Grigoriev, Daniel M GreenAbstract:ABSTRACTPurpose: We sought to assess accurately the full spectrum of end stage renal disease (ESRD) in Wilms Tumor survivors by combining the unique resources of the National Wilms Tumor Study Grou...
-
intravascular extension of Wilms Tumor
Annals of Surgery, 2001Co-Authors: Robert C Shamberger, Norman E. Breslow, Michael L Ritchey, Gerald M Haase, Tracy L Bergemann, Teri Loecheltyoshioka, Daniel M GreenAbstract:ObjectiveTo define the incidence and manifestations of and optimal therapy for children with intravascular extension of Wilms Tumor.MethodsChildren on a collaborative study of Wilms Tumor who had intravascular extension into the inferior vena cava (IVC) or atrium were identified. Surgical checklists
Michael L Ritchey - One of the best experts on this subject based on the ideXlab platform.
-
lymph node involvement in Wilms Tumor results from national Wilms Tumor studies 4 and 5
Journal of Pediatric Surgery, 2012Co-Authors: Kathleen Kieran, Daniel M Green, Michael L Ritchey, Robert C Shamberger, Elizabeth J. Perlman, Peter F Ehrlich, Jeffrey S Dome, James R Anderson, Andrew M. DavidoffAbstract:Purpose The aim of the study was to determine the prognostic impact of lymph node (LN) involvement and sampling in patients with Wilms Tumor (WT) and the minimum number of LNs needed for accurate staging.
-
Risk Factors for End Stage Renal Disease in Non-WT1-Syndromic Wilms Tumor
The Journal of Urology, 2011Co-Authors: Jane M. Lange, Susan M. Peterson, Janice R. Takashima, Yevgeny A. Grigoriev, Daniel M Green, Michael L Ritchey, Robert C Shamberger, J. Bruce Beckwith, Elizabeth J. Perlman, Norman E. BreslowAbstract:Purpose: We assessed risk factors for end stage renal disease in patients with Wilms Tumor without known WT1 related syndromes. We hypothesized that patients with characteristics suggestive of a WT1 etiology (early onset, stromal predominant histology, intralobar nephrogenic rests) would have a higher risk of end stage renal disease due to chronic renal failure. We predicted a high risk of end stage renal disease due to progressive bilateral Wilms Tumor in patients with metachronous bilateral disease.Materials and Methods: End stage renal disease was ascertained in 100 of 7,950 nonsyndromic patients enrolled in a National Wilms Tumor Study during 1969 to 2002. Risk factors were evaluated with cumulative incidence curves and proportional hazard regressions.Results: The cumulative incidence of end stage renal disease due to chronic renal failure 20 years after Wilms Tumor diagnosis was 0.7%. For end stage renal disease due to progressive bilateral Wilms Tumor the incidence was 4.0% at 3 years after diagnosi...
-
the management of synchronous bilateral Wilms Tumor a report from the national Wilms Tumor study group
Annals of Surgery, 2011Co-Authors: Thomas E Hamilton, Susan M. Peterson, Pedram Argani, Michael L Ritchey, Gerald M Haase, James R Anderson, Daniel Green, Robert C ShambergerAbstract:Objective To provide guidelines for future trials, we reviewed the outcomes of children with synchronous bilateral Wilms Tumors (BWT) treated on National Wilms Tumor Study-4 (NWTS-4).
-
bilateral Wilms Tumor with anaplasia lessons from the national Wilms Tumor study
Journal of Pediatric Surgery, 2006Co-Authors: Thomas E Hamilton, Daniel M Green, Pedram Argani, Paul E Grundy, Michael L Ritchey, Elizabeth J. Perlman, Robert C ShambergerAbstract:Abstract Purpose The purpose of this study was to evaluate whether initial diagnostic technique influenced the ability to identify anaplastic histology, to determine the time interval to diagnosis of anaplasia, and to delineate the incidence of discordant pathology in bilateral Wilms' Tumor. We hypothesized that delay in diagnosis of anaplasia could affect time to appropriate surgery and intensive multimodality therapy. Methods One hundred eight-nine children were enrolled in the fourth National Wilms' Tumor Study with synchronous bilateral Tumors, 27 of whom were eventually shown to have anaplastic histology. Initial diagnostic technique, time interval to diagnosis of anaplasia, and the incidence of discordant pathology were determined. Results Anaplasia was identified in 0 of 7 Tumors by core needle biopsy, 3 of 9 Tumors by open wedge biopsy, and in 7 of 9 cases by partial or complete nephrectomy. The mean duration of first chemotherapy regimen (DD or EE) was 20, 39, and 36 weeks, respectively, before anaplasia was identified at second surgery. Discordant pathology between bilateral Tumors was identified on final tissue diagnosis in 20 patients. Only 4 patients had anaplastic Tumors in both kidneys. Conclusions Core needle biopsy did not identify anaplasia in 7 of 7 children. Open biopsy or partial/complete nephrectomy identified anaplasia at initial diagnostic procedure in 10 of 18 children. Twenty of 24 patients at final tissue diagnosis had discordant pathology between the 2 kidneys. Earlier interval incisional biopsy or resection may identify anaplastic histology and limit the duration of chemotherapy targeted to favorable histology for children with bilateral Wilms' Tumor and anaplasia.
-
treatment of anaplastic histology Wilms Tumor results from the fifth national Wilms Tumor study
Journal of Clinical Oncology, 2006Co-Authors: Jeffrey S Dome, Norman E. Breslow, Paul E Grundy, Michael L Ritchey, Bruce J Beckwith, Elizabeth J. Perlman, Cecilia A Cotton, John A Kalapurakal, Marcio H Malogolowkin, Robert C ShambergerAbstract:Purpose An objective of the fifth National Wilms' Tumor Study (NWTS-5) was to evaluate the efficacy of treatment regimens for anaplastic histology Wilms' Tumor (AH). Patients and Methods Prospective single-arm studies were conducted. Patients with stage I AH were treated with vincristine and dactinomycin for 18 weeks. Patients with stages II to IV diffuse AH were treated with vincristine, doxorubicin, cyclophosphamide, and etoposide for 24 weeks plus flank/abdominal radiation. Results A total of 2,596 patients with Wilms' Tumor were enrolled onto NWTS-5, of whom 281 (10.8%) had AH. Four-year event-free survival (EFS) and overall survival (OS) estimates for assessable patients with stage I AH (n = 29) were 69.5% (95% CI, 46.9 to 84.0) and 82.6% (95% CI, 63.1 to 92.4). In comparison, 4-year EFS and OS estimates for patients with stage I favorable histology (FH; n = 473) were 92.4% (95% CI, 89.5 to 94.5) and 98.3% (95% CI, 96.4 to 99.2). Four-year EFS estimates for patients who underwent immediate nephrectom...
Robert C Shamberger - One of the best experts on this subject based on the ideXlab platform.
-
lymph node involvement in Wilms Tumor results from national Wilms Tumor studies 4 and 5
Journal of Pediatric Surgery, 2012Co-Authors: Kathleen Kieran, Daniel M Green, Michael L Ritchey, Robert C Shamberger, Elizabeth J. Perlman, Peter F Ehrlich, Jeffrey S Dome, James R Anderson, Andrew M. DavidoffAbstract:Purpose The aim of the study was to determine the prognostic impact of lymph node (LN) involvement and sampling in patients with Wilms Tumor (WT) and the minimum number of LNs needed for accurate staging.
-
Risk Factors for End Stage Renal Disease in Non-WT1-Syndromic Wilms Tumor
The Journal of Urology, 2011Co-Authors: Jane M. Lange, Susan M. Peterson, Janice R. Takashima, Yevgeny A. Grigoriev, Daniel M Green, Michael L Ritchey, Robert C Shamberger, J. Bruce Beckwith, Elizabeth J. Perlman, Norman E. BreslowAbstract:Purpose: We assessed risk factors for end stage renal disease in patients with Wilms Tumor without known WT1 related syndromes. We hypothesized that patients with characteristics suggestive of a WT1 etiology (early onset, stromal predominant histology, intralobar nephrogenic rests) would have a higher risk of end stage renal disease due to chronic renal failure. We predicted a high risk of end stage renal disease due to progressive bilateral Wilms Tumor in patients with metachronous bilateral disease.Materials and Methods: End stage renal disease was ascertained in 100 of 7,950 nonsyndromic patients enrolled in a National Wilms Tumor Study during 1969 to 2002. Risk factors were evaluated with cumulative incidence curves and proportional hazard regressions.Results: The cumulative incidence of end stage renal disease due to chronic renal failure 20 years after Wilms Tumor diagnosis was 0.7%. For end stage renal disease due to progressive bilateral Wilms Tumor the incidence was 4.0% at 3 years after diagnosi...
-
the management of synchronous bilateral Wilms Tumor a report from the national Wilms Tumor study group
Annals of Surgery, 2011Co-Authors: Thomas E Hamilton, Susan M. Peterson, Pedram Argani, Michael L Ritchey, Gerald M Haase, James R Anderson, Daniel Green, Robert C ShambergerAbstract:Objective To provide guidelines for future trials, we reviewed the outcomes of children with synchronous bilateral Wilms Tumors (BWT) treated on National Wilms Tumor Study-4 (NWTS-4).
-
bilateral Wilms Tumor with anaplasia lessons from the national Wilms Tumor study
Journal of Pediatric Surgery, 2006Co-Authors: Thomas E Hamilton, Daniel M Green, Pedram Argani, Paul E Grundy, Michael L Ritchey, Elizabeth J. Perlman, Robert C ShambergerAbstract:Abstract Purpose The purpose of this study was to evaluate whether initial diagnostic technique influenced the ability to identify anaplastic histology, to determine the time interval to diagnosis of anaplasia, and to delineate the incidence of discordant pathology in bilateral Wilms' Tumor. We hypothesized that delay in diagnosis of anaplasia could affect time to appropriate surgery and intensive multimodality therapy. Methods One hundred eight-nine children were enrolled in the fourth National Wilms' Tumor Study with synchronous bilateral Tumors, 27 of whom were eventually shown to have anaplastic histology. Initial diagnostic technique, time interval to diagnosis of anaplasia, and the incidence of discordant pathology were determined. Results Anaplasia was identified in 0 of 7 Tumors by core needle biopsy, 3 of 9 Tumors by open wedge biopsy, and in 7 of 9 cases by partial or complete nephrectomy. The mean duration of first chemotherapy regimen (DD or EE) was 20, 39, and 36 weeks, respectively, before anaplasia was identified at second surgery. Discordant pathology between bilateral Tumors was identified on final tissue diagnosis in 20 patients. Only 4 patients had anaplastic Tumors in both kidneys. Conclusions Core needle biopsy did not identify anaplasia in 7 of 7 children. Open biopsy or partial/complete nephrectomy identified anaplasia at initial diagnostic procedure in 10 of 18 children. Twenty of 24 patients at final tissue diagnosis had discordant pathology between the 2 kidneys. Earlier interval incisional biopsy or resection may identify anaplastic histology and limit the duration of chemotherapy targeted to favorable histology for children with bilateral Wilms' Tumor and anaplasia.
-
treatment of anaplastic histology Wilms Tumor results from the fifth national Wilms Tumor study
Journal of Clinical Oncology, 2006Co-Authors: Jeffrey S Dome, Norman E. Breslow, Paul E Grundy, Michael L Ritchey, Bruce J Beckwith, Elizabeth J. Perlman, Cecilia A Cotton, John A Kalapurakal, Marcio H Malogolowkin, Robert C ShambergerAbstract:Purpose An objective of the fifth National Wilms' Tumor Study (NWTS-5) was to evaluate the efficacy of treatment regimens for anaplastic histology Wilms' Tumor (AH). Patients and Methods Prospective single-arm studies were conducted. Patients with stage I AH were treated with vincristine and dactinomycin for 18 weeks. Patients with stages II to IV diffuse AH were treated with vincristine, doxorubicin, cyclophosphamide, and etoposide for 24 weeks plus flank/abdominal radiation. Results A total of 2,596 patients with Wilms' Tumor were enrolled onto NWTS-5, of whom 281 (10.8%) had AH. Four-year event-free survival (EFS) and overall survival (OS) estimates for assessable patients with stage I AH (n = 29) were 69.5% (95% CI, 46.9 to 84.0) and 82.6% (95% CI, 63.1 to 92.4). In comparison, 4-year EFS and OS estimates for patients with stage I favorable histology (FH; n = 473) were 92.4% (95% CI, 89.5 to 94.5) and 98.3% (95% CI, 96.4 to 99.2). Four-year EFS estimates for patients who underwent immediate nephrectom...
Shawn Yost - One of the best experts on this subject based on the ideXlab platform.
-
mutations in the transcriptional repressor rest predispose to Wilms Tumor
Nature Genetics, 2015Co-Authors: Shazia Mahamdallie, Sandra Hanks, Kristen L Karlin, Elizabeth R Perdeaux, Elise Ruark, Alexander Renwick, Anna Zachariou, Chad A Shaw, Emma Ramsay, Shawn YostAbstract:Nazneen Rahman and colleagues identify inactivating germline mutations in the gene encoding the transcriptional repressor REST in familial and non-familial cases of Wilms Tumor. The mutations cluster in the DNA-binding domain of REST and compromise REST transcriptional repression. Wilms Tumor is the most common childhood renal cancer1. To identify mutations that predispose to Wilms Tumor, we are conducting exome sequencing studies. Here we describe 11 different inactivating mutations in the REST gene (encoding RE1-silencing transcription factor) in four familial Wilms Tumor pedigrees and nine non-familial cases. Notably, no similar mutations were identified in the ICR1000 control series2 (13/558 versus 0/993; P < 0.0001) or in the ExAC series (13/558 versus 0/61,312; P < 0.0001). We identified a second mutational event in two Tumors, suggesting that REST may act as a Tumor-suppressor gene in Wilms Tumor pathogenesis. REST is a zinc-finger transcription factor that functions in cellular differentiation and embryonic development3,4. Notably, ten of 11 mutations clustered within the portion of REST encoding the DNA-binding domain, and functional analyses showed that these mutations compromise REST transcriptional repression. These data establish REST as a Wilms Tumor predisposition gene accounting for ∼2% of Wilms Tumor.
-
mutations in the transcriptional repressor rest predispose to Wilms Tumor
Nature Genetics, 2015Co-Authors: Shazia Mahamdallie, Sandra Hanks, Kristen L Karlin, Elizabeth R Perdeaux, Elise Ruark, Alexander Renwick, Anna Zachariou, Chad A Shaw, Emma Ramsay, Shawn YostAbstract:Wilms Tumor is the most common childhood renal cancer. To identify mutations that predispose to Wilms Tumor, we are conducting exome sequencing studies. Here we describe 11 different inactivating mutations in the REST gene (encoding RE1-silencing transcription factor) in four familial Wilms Tumor pedigrees and nine non-familial cases. Notably, no similar mutations were identified in the ICR1000 control series (13/558 versus 0/993; P < 0.0001) or in the ExAC series (13/558 versus 0/61,312; P < 0.0001). We identified a second mutational event in two Tumors, suggesting that REST may act as a Tumor-suppressor gene in Wilms Tumor pathogenesis. REST is a zinc-finger transcription factor that functions in cellular differentiation and embryonic development. Notably, ten of 11 mutations clustered within the portion of REST encoding the DNA-binding domain, and functional analyses showed that these mutations compromise REST transcriptional repression. These data establish REST as a Wilms Tumor predisposition gene accounting for ∼2% of Wilms Tumor.
