Related Experiment Video
Updated: May 10, 2026
12:03
In Vivo Model for Testing Effect of Hypoxia on Tumor Metastasis
Published on: December 9, 2016
Renal Ewing tumors
Summary
Renal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is rare but treatable with conventional ES protocols. Early diagnosis and guideline-adapted therapy improve outcomes for this rare kidney cancer.
Area of Science:
- Oncology
- Pediatric Oncology
- Rare Cancers
Background:
- Renal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is an exceptionally rare malignancy.
- Clinical presentation is often nonspecific, including abdominal pain, palpable mass, and hematuria.
- Diagnostic feasibility has improved with molecular detection of the EWS/ETS translocation.
Purpose of the Study:
- To analyze outcomes for patients with renal ES/PNET treated within established Ewing sarcoma study protocols.
- To evaluate the efficacy of current treatment strategies for this rare renal tumor.
Main Methods:
- Retrospective analysis of 24 patients with primary renal ES/PNET from the German GPOH Ewing sarcoma trials (1980-2009).
- Inclusion of a Medline search for additional renal ES/PNET cases.
- Median observation time of 3.71 years.
Main Results:
- Median patient age was 24.9 years, with 37.5% presenting with metastases.
- Tumor thrombi in renal vessels occurred in 56.2%; EWS/ETS translocation confirmed in 90.9%.
- Three-year overall survival (OS) was 0.80 and event-free survival (EFS) was 0.66 with combined chemotherapy and local control.
Conclusions:
- Renal ES/PNET warrants consideration in the differential diagnosis of renal tumors.
- Patients with renal ES/PNET demonstrate responsiveness to standard ES treatments.
- Emphasis on accurate diagnosis and adherence to guideline-based therapy is crucial for optimal patient management.
