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Updated: Jul 6, 2026
Modeling Chemotherapy Resistant Leukemia In Vitro
Published on: February 9, 2016
Current and emerging chemotherapy treatment strategies for Wilms tumor in North America
Eric J Gratias1, Jeffrey S Dome
1Division of Pediatric Hematology/Oncology, T.C. Thompson Children's Hospital, Chattanooga, Tennessee, USA. eric.gratias@erlanger.org
Insights
Wilms tumor, a common childhood kidney cancer, has a >90% survival rate for favorable histology cases. Treatment for anaplastic histology and relapsed Wilms tumor requires further research and international collaboration.
Area of Science:
- Pediatric Oncology
- Nephrology
- Cancer Genetics
Background:
- Wilms tumor is the predominant primary kidney cancer in children, with approximately 500 US diagnoses annually, primarily in those under 5 years old.
- Key prognostic indicators include disease stage, tumor histology, patient age, tumor weight, and specific chromosomal abnormalities (1p and 16q loss of heterozygosity).
Purpose of the Study:
- To review prognostic factors and treatment outcomes for childhood Wilms tumor.
- To highlight the ongoing challenges in treating anaplastic Wilms tumor and relapsed cases.
- To outline future research directions focusing on international collaboration for improved treatment strategies.
Main Methods:
- Analysis of data from five multicenter Wilms Tumor Study Group trials (1969-2002).
- Review of established prognostic factors influencing patient outcomes.
- Identification of treatment challenges and areas for future investigation.
Main Results:
- Overall survival rates exceed 90% for Wilms tumor patients with favorable histology.
- Anaplastic Wilms tumor histology presents a significant therapeutic challenge.
- Established prognostic factors guide current treatment approaches.
Conclusions:
- Wilms tumor treatment has achieved high cure rates for favorable histology, but challenges remain for anaplastic and relapsed cases.
- Future international collaborative studies are essential to optimize treatment strategies, minimize toxicity, and improve outcomes for all Wilms tumor patients.
- Advancing biologic understanding and novel therapeutic development are critical for improving the prognosis of refractory Wilms tumor cases.
Abstract:
Wilms tumor is the most common primary renal malignancy occurring in childhood. Approximately 500 children are diagnosed with Wilms tumor annually in the US alone, most of whom are aged <5 years. Several prognostic factors have been identified, including stage of disease, tumor histology, patient age, tumor weight, and tumor-specific loss of heterozygosity for chromosomes 1p and 16q. During the period from 1969 to 2002, the National Wilms Tumor Study Group coordinated five multicenter Wilms tumor studies. The overall survival rate for Wilms tumor has risen to >90% for patients with tumors of favorable histology. However, the treatment of patients with Wilms tumor with anaplastic histology remains challenging. The optimal treatment strategies for Wilms tumor in relapse will be studied via international collaboration in the near future. Goals of emerging studies include minimizing toxicity while maintaining the outstanding cure rates for patients with a good prognosis and, through advancing biologic understanding and developing novel therapeutic approaches, improving the prognosis for those patients in whom effective cure of their disease continues to elude physicians.
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