摘要
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.
出处
《中华小儿外科杂志》
CSCD
北大核心
2014年第2期81-84,共4页
Chinese Journal of Pediatric Surgery
基金
北京市优秀人才培养资助项目(2012D003034000021)
关键词
囊性
部分分化
WILMS瘤
Cystic
Partially differentiated
Wilms tumor