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 Childhood Renal Tumor: A Report from a Chinese Children’s Cancer Group

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Jiaoyang Cai ◽  
Ci Pan ◽  
Qin Lu ◽  
Jie Yan ◽  
Xiuli Ju ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Renal Tumor ◽  
Survival Rates ◽  
Renal Tumors ◽  
Group Model ◽  
Cooperative Group ◽  
Event Free Survival ◽  
Female Ratio ◽  
Free Survival ◽  
Favorable Histology

Here we investigated the establishment of multicenter cooperative treatment groups in China, as well as radiotherapy compliance and effectiveness among children with renal tumors. Medical records were reviewed for 316 children with renal tumors diagnosed by a multicenter cooperative group from 14 hospitals in China from 1998 to 2012. Median patient age was 29.5 months (range, 2–173 months old), and male-to-female ratio was 1.4 : 1. After a median follow-up of 22 months (range, 1–177 months), five-year event-free survival rates were 72% overall; 76.1% for favorable histology (251 cases); 59% for unfavorable histology (27 cases); and 91%, 75%, 71%, 53%, and 48.5%, respectively for Stages I, II, III, IV, and V. Following standardized criteria, radiation therapy was indicated for 153 patients, among whom five-year event-free survival was 72.8% for the 95 who received radiation and 24% for the 58 patients who did not. Our results are reasonable but can be further improved and show the feasibility of a multicenter cooperative group model for childhood renal tumor treatment in China. Radiation therapy is important for stage III and IV patients but remains difficult to implement in some parts of China. Government management departments and medical professionals must pay attention to this situation. This clinical trial is registered with ChiCTR-PRCH-14004372.

Pediatric Renal Tumors: Practical Updates for the Pathologist

2005 ◽  
Vol 8 (3) ◽  
pp. 320-338 ◽  
Author(s):  
Elizabeth J. Perlman
Keyword(s):  
Molecular Analysis ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Regional Lymph Node ◽  
Clear Cell Sarcoma ◽  
Considerable Change ◽  
Stage I ◽  
Renal Tumors ◽  
Favorable Histology ◽  
Pathologic Classification

Pediatric renal tumors were targeted by the National Wilms Tumor Study Group for 4 decades with extraordinary success. Within this historic context, this review provides a summary of the new Children's Oncology Group renal tumor protocols that will be opening in the very near future, focusing on their pathologic requirements. All renal tumors must first be registered on the Renal Tumor Classification and Banking Protocol, followed by registration on 1 of 4 primary therapeutic protocols based on histology, stage, and molecular analysis. This requires prompt submission of samples for molecular analysis and central pathologic review. Changes in staging criteria include classification of all tumor spillage as stage III, and requirement of regional lymph node evaluation for eligibility for stage I Wilms tumors (WTs) weighing less than 550 g in infants younger than 24 months and for stage I clear cell sarcoma. Patients with unilateral favorable histology WT with loss of heterozygosity for chromosomes 1p and 16q will receive more aggressive chemotherapy at each stage. Patients with bilateral WT and patients with diffuse hyperplastic perilobar nephroblastomatosis will be eligible for a novel therapeutic protocol requiring pathologic classification based on response of tumor to previous therapy. Stage I anaplastic WT will be targeted with more aggressive chemotherapy than in the past. For the first time, pediatric renal cell carcinoma will be eligible for a cooperative group protocol. All rhabdoid tumors outside the central nervous system will be eligible for a single protocol. In conclusion, these new protocols bring considerable change in their overall organization, in eligibility, and in therapy.

scholarly journals Predicting Relapse in Favorable Histology Wilms Tumor Using Gene Expression Analysis: A Report from the Renal Tumor Committee of the Children's Oncology Group

2009 ◽  
Vol 15 (5) ◽  
pp. 1770-1778 ◽  
Author(s):  
Chiang-Ching Huang ◽  
Samantha Gadd ◽  
Norman Breslow ◽  
Colleen Cutcliffe ◽  
Simone T. Sredni ◽  
...  
Keyword(s):  
Gene Expression ◽  
Expression Analysis ◽  
Gene Expression Analysis ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Oncology Group ◽  
Favorable Histology

scholarly journals Challenges on participation in a cooperative group of childhood renal tumors in Brasil

2020 ◽  
Vol 66 (3) ◽  
pp. 284-289
Author(s):  
Lucian S. Viana ◽  
Neimar de Paula Silva ◽  
Nathalie V. Balmant ◽  
Paulo A. Faria ◽  
Marceli O. Santos ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Cancer Registries ◽  
Geographic Region ◽  
Renal Tumors ◽  
Trial Participation ◽  
Cooperative Group ◽  
Cooperative Groups ◽  
Accrual Rate

SUMMARY OBJECTIVE Children with renal tumors included in clinical trials have significantly better outcomes. In Brasil, the enrollment of patients in clinical trials remains challenging. Here we aimed to describe participation accrual in the Brazilian Wilms Tumor Study Group (BWTSG) and to identify barriers to trial registration of children with renal tumors. METHODS We determined the numbers of renal tumor diagnoses in 105 hospital-based cancer registries from 2001-2009. We then compared these totals with the numbers of renal tumor cases registered in the BWTSG from the same hospitals during the same time period. We also invited members of the Brazilian Pediatric Oncology Society to complete a 5-point Likert-type scale questionnaire regarding their opinions of the importance of participation in cooperative group trials. RESULTS The accrual rate of patient participation per hospital varied from 25% to 76%, and was highest in the South region. The accrual rate of hospital participation also varied according to the region (20-31%) and was highest in the Southeast region. For the questionnaire regarding the importance of participation in cooperative groups, the responses showed an agreement of >75% on 10 of the 13 statements. CONCLUSION Our results demonstrated low accrual of participation in a cooperative group trial in Brasil. We identified variations in registration rates according to geographic region and hospital, which may help targeted efforts to increase registration rates. The survey responses demonstrated that colleagues understand the importance of trial participation.

Wilms Tumor

2018 ◽  
Author(s):  
Graciela Argote-Romero
Keyword(s):  
Wilms Tumor ◽  
Incidental Finding ◽  
Abdominal Mass ◽  
Disease Free Survival ◽  
Von Willebrand Disease ◽  
Renal Tumors ◽  
Intravenous Contrast ◽  
Favorable Histology ◽  
Disease Free Survival Rate ◽  
Von Willebrand

Wilms tumor, known as well as nephroblastoma, is the most common primary malignant renal tumor in children. Over 95% of all renal tumors in patients under the age of 15 are Wilms tumors. The mean age at the time of diagnosis is 3.5 years. Wilms tumors are usually an incidental finding, a large abdominal mass discovered by a family member or pediatrician. Hematuria and hypertension can be present at the time of initial diagnosis. Up to 8% of the patients will have von Willebrand disease; therefore, all patients should have baseline coagulation studies. All patients should have either computed tomography of the abdomen and pelvis with oral and intravenous contrast or magnetic resonance imaging of the abdomen and pelvis with gadolinium. Treatment includes radical nephrectomy, chemotherapy, and, in some cases, radiotherapy. Emergency surgery is rarely. The disease-free survival rate is 86% for favorable-histology tumors and 64% for anaplastic tumors.

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.

Cancer in Neonates: The Experience at The Children's Hospital of Philadelphia

PEDIATRICS ◽  
1982 ◽  
Vol 70 (3) ◽  
pp. 409-413
Author(s):  
Gordon B. Gale ◽  
Giulio J. D'Angio ◽  
Antonia Uri ◽  
Jane Chatten ◽  
C. Everett Koop
Keyword(s):  
Cancer Research ◽  
Radiation Therapy ◽  
Wilms Tumor ◽  
Surgical Excision ◽  
Careful Monitoring ◽  
Actuarial Survival ◽  
Parotid Carcinoma ◽  
Complete Surgical Excision ◽  
Long Term Survivors

Among 22 neonates treated at the Children's Cancer Research Center of Philadelphia, 11 had neuroblastoma, which in two cases was widely metastatic. There were three infants with teratoma, three with sarcoma, three with leukemia, one with Wilms' tumor, and one with parotid carcinoma. Nine of eleven patients (82%) are long-term survivors following complete surgical excision of tumor, whereas only one of eight (13%) has survived following incomplete surgical excision. All three neonates with leukemia died. The overall two-year actuarial survival is 45% (10/22). The problems associated with treating neonates with chemotherapy, radiation therapy, or both are especially difficult because of the immaturity of the organs and structures. Surgical excision alone has been the treatment of choice for solid tumors. Chemotherapy or radiation therapy, when indicated, require careful monitoring for both acute toxicities and potential long-term morbidities.

scholarly journals A case of Wilms tumor with right ventricular extension of tumor thrombus

2017 ◽  
Vol 4 (1) ◽  
pp. 60
Author(s):  
Mustafa Çakan ◽  
Ayşe Gülnur Tokuç ◽  
Kıvılcım Karadeniz Cerit ◽  
Koray Ak ◽  
Rabia Ergelen
Keyword(s):  
Inferior Vena Cava ◽  
Right Ventricle ◽  
Right Atrium ◽  
Tumor Thrombus ◽  
Inferior Vena ◽  
Renal Tumor ◽  
Wilms Tumor ◽  
Vena Cava ◽  
Renal Tumors ◽  
Tumor Extension

Primary renal tumors comprise 6% of all childhood cancers. Wilms tumor is the most common primary renal tumor in pediatric age group and the peak age of diagnosis is 3-4 years. In 10% of cases tumor extension into hepatic vein and inferior vena cava can be seen. But tumor extension into whole inferior vena cava, right atrium and right ventricle is only seen in less than 1% of patients. A 2-year-old girl was admitted to the hospital because of abdominal distension that was noticed by the parents two weeks ago. Imaging studies revealed that she had a mass at the right renal lodge which was favoring to Wilms tumor and on thorax tomography tumor thrombus was seen in the whole inferior vena cava, right atrium and right ventricle. Neoadjuvant chemotherapy was given for 7 weeks. On the 8th week of diagnosis, under cardiopulmonary bypass, surgical operation by pediatric and cardiovascular surgery teams for primary renal tumor and for cavo-atrial tumor thrombus was performed. Pathological examination of the mass was reported as stage 3 diffuse anaplastic Wilms tumor. The patient completed 24 weeks of chemotherapy protocol and she is being followed for 15 months without any morbidity. We present our case to emphasize the importance of multidisciplinary approach in Wilms tumor with cardiac extension.

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