Impact of Radiation Therapy on Patterns of Relapse and Survival in Children with Stage III Favorable Histology Wilms Tumor

2018 ◽  
Vol 102 (3) ◽  
pp. S52-S53
Author(s):  
J.A. Kalapurakal ◽  
A.C. Paulino ◽  
Y.Y. Chi ◽  
Y. Kim ◽  
L. Ji ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Wilms Tumor ◽  
Stage Iii ◽  
Favorable Histology

scholarly journals Augmentation of Therapy for Combined Loss of Heterozygosity 1p and 16q in Favorable Histology Wilms Tumor: A Children’s Oncology Group AREN0532 and AREN0533 Study Report

2019 ◽  
Vol 37 (30) ◽  
pp. 2769-2777 ◽  
Author(s):  
David B. Dix ◽  
Conrad V. Fernandez ◽  
Yueh-Yun Chi ◽  
Elizabeth A. Mullen ◽  
James I. Geller ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Loss Of Heterozygosity ◽  
Wilms Tumor ◽  
Adverse Outcomes ◽  
Stage I ◽  
Stage Iii ◽  
Rank Test ◽  
Favorable Histology ◽  
Grade 3

PURPOSE In National Wilms Tumor Study 5 (NWTS-5), tumor-specific combined loss of heterozygosity of chromosomes 1p and 16q (LOH1p/16q) was associated with adverse outcomes in patients with favorable histology Wilms tumor. The AREN0533/AREN0532 studies assessed whether augmenting therapy improved event-free survival (EFS) for these patients. Patients with stage I/II disease received regimen DD4A (vincristine, dactinomycin and doxorubicin) but no radiation therapy. Patients with stage III/IV disease received regimen M (vincristine, dactinomycin, and doxorubicin alternating with cyclophosphamide and etoposide) and radiation therapy. METHODS Patients were enrolled through the AREN03B2 Biology study between October 2006 and October 2013; all underwent central review of pathology, surgical reports, and imaging. Tumors were evaluated for LOH1p/16q by microsatellite testing. EFS and overall survival were compared using the log-rank test between NWTS-5 and current studies. RESULTS LOH1p/16q was detected in 49 of 1,147 evaluable patients with stage I/II disease (4.27%) enrolled in AREN03B2; 32 enrolled in AREN0532. LOH1p/16q was detected in 82 of 1,364 evaluable patients with stage III/IV disease (6.01%) in AREN03B2; 51 enrolled in AREN0533. Median follow-up for 83 eligible patients enrolled in AREN0532/0533 was 5.73 years (range, 2.84 to 9.63 years). The 4-year EFS for patients with stage I/II and stage III/IV disease with LOH1p/16 was 87.3% (95% CI, 75.1% to 99.5%) and 90.2% (95% CI, 81.8% to 98.6%), respectively. These results are improved compared with the NWTS-5 updated 4-year EFS of 68.8% for patients with stage I/II disease ( P = .042), and 61.3% for patients with stage III/IV disease ( P = .001), with trends toward improved 4-year overall survival. The most common grade 3 or higher nonhematologic toxicities with regimen M were febrile neutropenia (39.2%) and infections (21.6%). CONCLUSION Augmentation of therapy improved EFS for patients with favorable histology Wilms tumor and LOH1p/16q compared with the historical NWTS-5 comparison group, with an expected toxicity profile.

scholarly journals Outcome and Prognostic Factors in Stage III Favorable-Histology Wilms Tumor: A Report From the Children’s Oncology Group Study AREN0532

2018 ◽  
Vol 36 (3) ◽  
pp. 254-261 ◽  
Author(s):  
Conrad V. Fernandez ◽  
Elizabeth A. Mullen ◽  
Yueh-Yun Chi ◽  
Peter F. Ehrlich ◽  
Elizabeth J. Perlman ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Lymph Node ◽  
Residual Disease ◽  
Wilms Tumor ◽  
Stage Iii ◽  
Lymph Node Status ◽  
Oncology Group ◽  
Favorable Histology ◽  
Node Status

Background The National Wilms Tumor Study (NWTS) approach to treating stage III favorable-histology Wilms tumor (FHWT) is Regimen DD4A (vincristine, dactinomycin, and doxorubicin) and radiation therapy. Further risk stratification is required to improve outcomes and reduce late effects. We evaluated clinical and biologic variables for patients with stage III FHWT without combined loss of heterozygosity (LOH) at chromosomes 1p and 16q treated in the Children’s Oncology Group protocol AREN0532. Methods From October 2006 to August 2013, 588 prospectively treated, centrally reviewed patients with stage III FHWT were treated with Regimen DD4A and radiation therapy. Tumor LOH at 1p and 16q was determined by microsatellite analysis. Ineligible patients (n = 5) and those with combined LOH 1p/16q (n = 40) were excluded. Results A total of 535 patients with stage III disease were studied. Median follow-up was 5.2 years (range, 0.2 to 9.5). Four-year event-free survival (EFS) and overall survival estimates were 88% (95% CI, 85% to 91%) and 97% (95% CI, 95% to 99%), respectively. A total of 58 of 66 relapses occurred in the first 2 years, predominantly pulmonary (n = 36). Eighteen patients died, 14 secondary to disease. A better EFS was associated with negative lymph node status ( P < .01) and absence of LOH 1p or 16q ( P < .01), but not with gross residual disease or peritoneal implants. In contrast, the 4-year EFS was only 74% in patients with combined positive lymph node status and LOH 1p or 16q. A total of 123 patients (23%) had delayed nephrectomy. Submitted delayed nephrectomy histology showed anaplasia (n = 8; excluded from survival analysis); low risk/completely necrotic (n = 7; zero relapses), intermediate risk (n = 63; six relapses), and high-risk/blastemal type (n=7; five relapses). Conclusion Most patients with stage III FHWT had good EFS/overall survival with DD4A and radiation therapy. Combined lymph node and LOH status was highly predictive of EFS and should be considered as a potential prognostic marker for future trials.

Outcome and prognostic factors in stage III favorable histology Wilms tumor (FHWT): A report from the Children’s Oncology Group (COG) study AREN0532.

2015 ◽  
Vol 33 (15_suppl) ◽  
pp. 10010-10010 ◽  
Author(s):  
Conrad Vincent Fernandez ◽  
Elizabeth Anne Mullen ◽  
Peter F. Ehrlich ◽  
John A. Kalapurakal ◽  
Geetika Khanna ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Wilms Tumor ◽  
Stage Iii ◽  
Oncology Group ◽  
Favorable Histology

Radiotherapy omitted in the treatment of selected children under 3 years of age with stage III favorable histology Wilms tumor

1998 ◽  
Vol 31 (3) ◽  
pp. 150-152 ◽  
Author(s):  
A. Pachnis ◽  
J. Pritchard ◽  
M. Gaze ◽  
G. Levitt ◽  
A. Michalski
Keyword(s):  
Wilms Tumor ◽  
Stage Iii ◽  
Favorable Histology

scholarly journals Radiation therapy for favorable histology Wilms tumor: Prevention of flank recurrence did not improve survival on National Wilms Tumor Studies 3 and 4

2006 ◽  
Vol 65 (1) ◽  
pp. 203-209 ◽  
Author(s):  
Norman E. Breslow ◽  
J. Bruce Beckwith ◽  
Gerald M. Haase ◽  
John A. Kalapurakal ◽  
Michael L. Ritchey ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Wilms Tumor ◽  
Favorable Histology ◽  
Tumor Studies ◽  
Tumor Prevention

scholarly journals Wilms Tumor (Nephroblastoma), Version 2.2021, NCCN Clinical Practice Guidelines in Oncology

2021 ◽  
Vol 19 (8) ◽  
pp. 945-977
Author(s):  
Frank Balis ◽  
Daniel M. Green ◽  
Clarke Anderson ◽  
Shelly Cook ◽  
Jasreman Dhillon ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Renal Tumors ◽  
Nccn Guidelines ◽  
Favorable Histology ◽  
Tumor Focus ◽  
Appropriate Treatment ◽  
Pediatric Oncologist

The NCCN Guidelines for Wilms Tumor focus on the screening, diagnosis, staging, treatment, and management of Wilms tumor (WT, also known as nephroblastoma). WT is the most common primary renal tumor in children. Five-year survival is more than 90% for children with all stages of favorable histology WT who receive appropriate treatment. All patients with WT should be managed by a multidisciplinary team with experience in managing renal tumors; consulting a pediatric oncologist is strongly encouraged. Treatment of WT includes surgery, neoadjuvant or adjuvant chemotherapy, and radiation therapy (RT) if needed. Careful use of available therapies is necessary to maximize cure and minimize long-term toxicities. This article discusses the NCCN Guidelines recommendations for favorable histology WT.

scholarly journals Clinicopathologic Findings Predictive of Relapse in Children With Stage III Favorable-Histology Wilms Tumor

2013 ◽  
Vol 31 (9) ◽  
pp. 1196-1201 ◽  
Author(s):  
Peter F. Ehrlich ◽  
James R. Anderson ◽  
Michael L. Ritchey ◽  
Jeffrey S. Dome ◽  
Daniel M. Green ◽  
...  
Keyword(s):  
Relative Risk ◽  
Residual Disease ◽  
Wilms Tumor ◽  
Prognostic Significance ◽  
Stage Iv ◽  
Stage Iii ◽  
Apparent Difference ◽  
Favorable Histology ◽  
Stage Iv Disease ◽  
Microscopic Residual Disease

Purpose Stage III designation in NWTS-5 (National Wilms Tumor Study–5) was determined by four pathologic criteria: positive lymph nodes (LNs), peritoneal implants, residual disease, and tumor rupture. The objective of this study was to determine the prognostic significance of each of the stage III criteria. Patients and Methods Children with stage III Wilms tumor (WT) treated in NWTS-5 were assessed for event-free (EFS) and overall survival (OS). Sites of relapse and molecular status of tumors are reported. EFS and OS are reported 8 years after diagnosis. Results There were 569 patients with local stage III favorable-histology (FH) WT in this analysis, of whom 109 had overall stage IV disease. LN involvement alone was the most frequent criterion for stage III designation (38%), followed by microscopic residual disease alone (20%), microscopic residual disease and LN involvement (14%), and spill or soilage alone (9%). The 8-year EFS and OS estimates for all patients with local stage III FHWT were 82% and 91%, respectively. Multivariate analysis demonstrated that both LN involvement (relative risk, 1.89; P = .005) and microscopic residual disease (relative risk, 1.87; P = .007) were predictive of EFS, and OS results were similar. There was no apparent difference in pattern of relapse according to stage III subtype. The rate of loss of heterozygosity was higher (6%) for those with positive LNs than for those without (2%; P = .05). Conclusion LN involvement and microscopic residual are the stage III criteria highly predictive of EFS and OS for patients with stage III FHWT. It is possible that in future studies, patients with different stage III criteria may receive different therapies.

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