畸胎瘤样肾母细胞瘤治疗体会及文献回顾  

Diagnosis and treatment of teratoid Wilms＇ tumor with a literature review

作　　者：吴伟[1] 刘江斌[1] 徐伟珏[1] 余胜华 吕志宝[1] 

机构地区：[1]上海交通大学附属儿童医院,上海市儿童医院外科,200040

出　　处：《中华小儿外科杂志》2017年第10期749-752,共4页Chinese Journal of Pediatric Surgery

基　　金：上海市卫计委课题（12411952405）

摘　　要：目的探讨儿童畸胎瘤样肾母细胞瘤临床诊断和治疗方法，提高临床对该病的认识。方法回顾我院2007年至2016年间诊治的4例儿童畸胎瘤样肾母细胞瘤，男2例，女2例，年龄2-40个月，平均年龄21个月；原发灶：右肾3例，左肾1例；根据cOG分期为Ⅰ、Ⅱ、Ⅲ、Ⅳ期（肺及骨髓）各1例，分析其临床表现、诊断及治疗方法。结果4例患儿均行肾脏肿瘤根治性切除术，其中Ⅲ、Ⅳ期患儿术前行化疗再行根治术；3例术后辅以化疗，术后复发1例（化疗不敏感）；3例早中期患儿预后可（2例随访时间超9年，1例随访3年），1例Ⅳ患儿再次手术后化疗加放疗中。结论儿童畸胎瘤样肾母细胞瘤是肾母细胞瘤的罕见病理类型，临床症状通常难与经典肾母细胞瘤鉴别，主要依靠术前影像学及病理诊断，患侧肾脏肿瘤根治性切除术为主要治疗手段，早中期TWT患儿预后良好，至于保肾肿瘤切除术在该类型肾母细胞瘤的应用，暂未见文献报道，本组病例数较少，也未涉及。对于晚期TWT患儿，可能因为其独特的病理特征，其预后可能比经典肾母细胞瘤更差。Objective To its public awareness. Methods explore the diagnosis and treatment of teratoid Wilms＂ tumor and raise Four hospitalized patients of teratoid Wilms＂ tumor between 2007 to 2016 were evaluated retrospectively. There were 2 males and 2 females with a mean age of 21 months. The involved side was right （n = 3） and left （n = 1）. Based upon the staging scheme of Children＇s Oncology Group,there were 1 case each of types I , II ,III, IV （lung ＆ bone marrow）. The clinical manifestations,diagnosis and treatment were analyzed. Results Radical nephrectomy was performed. Two of them received perioperative chemotherpy. Three patients received chemotherapy and 1 case had postoperative recurrence. The prognosis of 3 patients was decent. Two patients were followed up for over 9 years while another was tracked for 3 years. One patient of stage IV received chemoradiotherapy after reoperation. Conclusions Teratoid Wilms＂ tumor is an unusual variant of nephroblastoma with a predominance of heterologous tissue. And its definite diagnosis is dependent upon imaging and pathological examination. Surgical resection remains a major treatment.

关 键 词：畸胎瘤样 肾母细胞瘤 肾脏肿瘤根治术 

分 类 号：R737.11[医药卫生—肿瘤]