Renal clear cell sarcoma in children: A case report

Clear cell sarcoma of the kidney (CCSK), a rare renal interstitial tumor in children, poses a notable threat to the physical and mental health of children because of its highly aggressive nature, tendency to compress surrounding tissue and potential for metastasis. The present study reports two cases of pediatric CCSK treated at the Peking University First Hospital Ningxia Women and Children's Hospital (Ningxia Hui Autonomous Region Maternal and Child Health Hospital, Ningxia, China; July 2024-2025.7). Both patients were female infants aged 1 year. Upon initial admission, they were misdiagnosed with nephroblastoma and received radical nephrectomy and vincristine chemotherapy. Pathological consultation and advanced molecular sequencing identified specific expression of the BCL6 corepressor gene in patient tumor tissue, leading to a definitive diagnosis of CCSK. Furthermore, the present study aimed to provide an overview of the aberrant genes associated with CCSK to provide a useful reference for future research on the pathogenesis of CCSK and improvement of molecular pathology.