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Renal cell carcinoma in children: a clinicopathologic study
Paolo Indolfi1, Monica Terenziani, Fiorina Casale
1Pediatric Oncology Service-Pediatric Department II, University of Napoli, Napoli, Italy. paolo.indolfi@unina2.it
Summary
Renal cell carcinoma (RCC) is rare in children, with prognosis significantly linked to disease stage. Localized pediatric RCC may be cured by nephrectomy alone, but advanced stages require further investigation for effective treatments.
Area of Science:
- Pediatric Oncology
- Nephrology
- Cancer Research
Background:
- Renal cell carcinoma (RCC) is an uncommon malignancy in pediatric populations.
- Understanding prognostic factors and treatment outcomes is crucial for improving survival rates in children with RCC.
Purpose of the Study:
- To identify prognostic factors, evaluate treatment strategies, and determine outcomes for pediatric patients diagnosed with renal cell carcinoma (RCC).
Main Methods:
- A retrospective analysis of 41 pediatric patients (median age 124 months) diagnosed with RCC between 1973 and 2001.
- Data collected included clinical presentation, surgical details, pathology, and treatment summaries from 11 Italian centers.
Main Results:
- Histology was predominantly nonpapillary (82.4%). Disease stages ranged from I (43.9%) to IV (21.9%), with one case of bilateral involvement.
- The most significant prognostic factor identified was the disease stage at diagnosis. Overall and event-free survival at 20 years were approximately 54.9% and 53.5%, respectively.
Conclusions:
- Pediatric RCC, while rare, presents differently than in adults but shares similar outcomes.
- Nephrectomy alone may suffice for localized disease; further research is needed for adjuvant therapies in advanced stages (Stage III).
- Alternative therapeutic approaches are suggested for patients with advanced or metastatic renal cell carcinoma.