Abstract
Renal cell carcinoma (RCC) is an extremely rare cancer in pediatric, accounting for 0.1% to 0.3% of all pediatric malignancies. Due to its low incidence in this age group, literature on pediatric RCC is limited. We present a case of localized RCC in pediatric patient treated with nephroureterectomy and lymphadenectomy. A 14-year-old girl with a 2-year history of red urine, blood clots, and left flank pain underwent physical examination, revealing left flank tenderness. Laboratory results showed anemia, leukocytosis, elevated LDH, and erythrocytes in urine. Imaging revealed a hyperechoic area in the left kidney's lower pole (4.0 × 2.8 cm). Contrast abdominal CT scan indicated a solid mass (5.4 × 3.8 × 3.2 cm) in left renal pelvis extending to the minor calyx and severe hydronephrosis. The patient was diagnosed with suspected cT3N0M0 left upper tract urothelial carcinoma and underwent cystoscopy, bladder cuff excision, and left radical nephroureterectomy with paraaortic lymph node dissection. Pathological analysis revealed clear cell type renal cell carcinoma, WHO ISUP grade IV, extending to the perirenal fat, with tumor-free ureteral resection margins, and no paraaortic lymph node metastasis. Follow-up on postoperative period showed a satisfactory outcome. In cases of pediatric localized RCC, surgical therapy, including radical nephrectomy or nephron-sparing surgery (NSS), can be a preferred treatment for small tumor volumes. This approach preserves kidney function and is generally considered safe for achieving tumor-free margins.