肾母细胞瘤是儿童最常见的原发性肾脏恶性肿瘤。完整切除肿瘤和精确判断肿瘤扩散程度是决定肿瘤准确分期和后续治疗的关键。保留肾单位手术治疗双侧肾母细胞瘤安全且有效。双侧肾母细胞瘤、肿瘤并下腔静脉和心房瘤栓以及术前影像学检查容积大于1 000 mL的肿瘤,接受新辅助化疗后再手术,可降低手术并发症的风险。肾母细胞瘤新的治疗方案是在细化危险分层系统的基础上,进行个体化治疗,以保持较高治愈率,进一步降低化疗和放疗的毒性作用。 Wilms tumor is the most common primary renal malignancy in children. Complete resection of the tumor and accurately determine the degree of tumor spread is the key to determine the exact stage of the tumor and follow-up treatment. Surgical treatment of nephrotic nephroblastoma is safe and effective. Bilateral nephroblastoma, tumors with inferior vena cava and atrial tumor suppository, and preoperative imaging examination of tumors with a volume of more than 1 000 mL. Reoperation after neoadjuvant chemotherapy can reduce the risk of surgical complications. The new treatment of nephroblastoma is to refine the risk stratification system based on individualized treatment to maintain a high cure rate and further reduce the toxic effects of chemotherapy and radiotherapy.