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Congenital mesoblastic nephroma: a single-center retrospective study
Jian Li1, Mengwen Yan1, Tianjiao Hu2
1Department of Oncology, Children's Hospital of Nanjing Medical University, Nanjing, China.
Translational Pediatrics
|March 11, 2026
Summary
Congenital mesoblastic nephroma (CMN) is a rare infant kidney tumor. While surgery offers good outcomes, molecularly atypical cases present challenges, necessitating further research for optimal management.
Area of Science:
- Pediatric Oncology
- Nephrology
- Molecular Pathology
Background:
- Congenital mesoblastic nephroma (CMN) is a rare renal tumor in neonates and infants.
- Standardized treatment guidelines for CMN are lacking.
Purpose of the Study:
- To summarize clinical features, treatment strategies, and outcomes of CMN patients.
- To inform clinical practice for congenital mesoblastic nephroma.
Main Methods:
- Retrospective review of nine pathologically and molecularly confirmed CMN cases.
- Analysis of demographic, clinical, imaging, histological, and molecular data.
- Evaluation of treatment approaches and follow-up outcomes.
Main Results:
- Most patients (90%) presented with an abdominal mass; median age at diagnosis was 2.5 months.
- Common imaging findings included mixed cystic-solid masses with heterogeneous enhancement.
- Molecular analysis identified ETV6::NTRK3, TPM3::NTRK1, and EGFR-KDD fusions in some cases.
- Surgery achieved a 100% resection rate; salvage chemotherapy showed limited efficacy in relapsed cases.
- Overall survival was 88.9% and event-free survival was 77.8% after median follow-up of 36 months.
Conclusions:
- CMN exhibits clinical and molecular heterogeneity.
- Favorable outcomes are common post-surgery, but relapsed cases pose management challenges.
- Larger collaborative studies are needed to define prognostic factors and optimal CMN management.
