囊性部分分化性肾母细胞瘤的诊治

Diagnosis and therapy of cystic partially differentiated nephroblastoma

目的总结囊性部分分化性肾母细胞瘤的临床、病理特点，提高对该病的认识。方法回顾分析我院收治6例囊性部分分化性肾母细胞瘤的临床资料，包括患儿年龄、临床表现、影像检查、病理、治疗和预后。结果6例患儿，男4例，女2例，年龄4个月至3岁5个月，平均18个月，左侧2例，右侧3例，双侧1例。腹部包块5例，B型超声检查偶然发现1例，术前均行B型超声和增强CT检查。2例3侧行肿瘤剜除术，4例行瘤肾切除术。均经病理诊断证实。术后随访6个月至7年，未见肿瘤复发，保留肾脏的2例3侧残肾功能良好。结论囊性部分分化性肾母细胞瘤是肾母细胞瘤的少见特殊亚型，低度恶性，预后良好。Ⅰ期可单纯手术治疗，Ⅱ期以上手术＋化疗，条件允许可行保留肾脏的肿瘤剜除或部分肾切除。

Objective To explore the characteristic clinical cystic partially differentiated nephroblastoma （CPDN）. Methods profiles and treatment modalities of A retrospective study was conduc- ted for 6 CPDN patients. Their clinical data were collected to analyze clinical presentations, imaging findings and treatment modalities. Results There were 4 males and 2 females with a mean age of 18 （4-36） months. Abdominal mass was the main clinical presentation. Ultrasound and computed tomo- graphy （CT） were performed. Two patients underwent trilateral tumor enucleation via a nephron- sparing approach and another 4 had nephrectomy. Postoperative pathology confirmed CPDN in all 6 cases. During a follow-up period of 6 months to 7 years, they had no evidence of recurrence. Conclusions CPDN is a rare differentiated variant of WiIm＇s tumor with low malignant potentials. Stage Ⅰ tumor should be treated with nephrectomy alone while stage Ⅱ undergoes nephrectomy fol- lowed by postoperative vincristine and dactinomycin chemotherapy.