To evaluate the feasibility and outcomes of robotic-assisted laparoscopic radical nephrectomy for pediatric renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion.

A retrospective study was performed of patients who underwent robotic assisted laparoscopic approach for renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion at our institution between 2017 to 2019. The epidemiological character, clinical data, histologic findings, image data and outcomes of these cases were reviewed together with the related literatures.

A 4-year-old girl and a 6-year-old boy with gross hematuria were diagnosed with left renal tumor by ultrasound, CT and MRI. Two patients underwent robotic assisted laparoscopic radical nephrectomy without conversion to open surgery. Grossly, The tumor was soft and the cut surface was brown. Microscopically, the tumor predominantly composed of clear cells. The tumor cells were rich of eosinophilic granular cytoplasm. Immunohistochemically, the tumor cells displayed as TEF3(+), CD10(+), CK(-), WT1(-), Vimentin(-), EMA(-), Ki-67(+2%), Bcl-2(+), CD34(-), S100(-), DES(-), CD99(-) and INI-1(+). The pathological diagnosis was renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion. There was no perioperative complications, and all patients were survived without recurrence or metastasis during the follow-up period.

Robotic assisted laparoscopic radical nephrectomy can be safely and effectively performed on early staged pediatric renal cell carcinoma associated with Xp11.2 translocation/TFE3 gene fusion.