Abstract: Objective To explore the expressions of protein signaling regulator 4（RGS 4），chemokine receptor chemokine receptor 4（CXCR4），topoisomerase α（TOPOⅡα）in pediatric Wilms tumor tissues and their relationships with clinicopathological characteristics and prognosis. Methods The cancer tissues of 40 children with nephroblastoma treated by surgery in The second Affiliated Hospital of Hainan Medical University from January 2017 to January 2020 were selected as the study subjects，40 with adjacent tissues > 4 cm away from the edge of cancer tissue kept as controls. The relationship between the expressions of RGS 4 protein，CXCR 4 and TOPOⅡα and the clinicopathological characteristics and survival time in children with Wilms′ tumor. Results Compared with the paracancer tissues，the high expression rates of TOPOⅡα and CXCR4 in Wilms tumor tissues were significantly increased，while the high expression rates of RGS4 protein were significantly decreased（P < 0.05）. RGS4 protein was negatively correlated with lymph node metastasis，pathological type，recurrence and metastasis，and TNM stage. CXCR4 and TOPOⅡα were positively correlated with them（P < 0.05）. RGS4，CXCR4 and TOPOⅡα were correlated with recurrence and metastasis，pathological type，lymph node metastasis and TNM stage（P < 0.05）. Those with high expression of RGS4 protein had higher overall survival rate，while those with high expressions of CXCR4 and TOPOⅡα had lower overall survival rate. Conclusion The expressions of RGS4 protein，CXCR4 and TOPOⅡα in pediatric tissues are closely associated with the clinicopathological characteristics and prognosis survival. 

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