中文摘要：

目的 探讨胎儿横纹肌瘤型（fetal rhabdomyomatous nephroblastoma,FRN）肾母细胞瘤的诊治、病理特点及预后.方法 回顾分析2000年1月至2015年10月收治的14例胎儿横纹肌瘤型肾母细胞瘤患儿的临床资料.男8例,女6例;年龄7个月～8岁8个月,平均23个月.肿瘤位于左侧3例,右侧5例,双侧病变6例.临床分期：Ⅰ期2例,Ⅱ期1例,Ⅲ期5例,Ⅴ期6例.临床表现为腹部包块11例,血尿、腹痛、呕吐各1例.B超检查示肿瘤呈中低不均混合回声,内可见囊腔或完全为实性.CT检查多提示肿瘤源于肾脏一极,表现为强化不均匀的实性肿块,瘤内可见坏死、囊变,少数有出血、钙化.B超及增强CT检查较典型肾母细胞瘤无特殊表现.9例接受术前化疗,瘤体均无缩小.单侧肿瘤患者均接受根治性肾切除术,4例双侧病变者行肾部分切除或单纯肿瘤切除术.结果 病理检查镜下可见大部分（70％以上）向横纹肌分化的肌母细胞,免疫组化染色检查示无特异性标志物,Desmin和Myogenin多为阳性.病理诊断：8例为单侧FRN,2例为双侧FRN,3例为一侧FRN另一侧肾母细胞瘤病,1例为一侧FRN另一侧间叶型肾母细胞瘤.14例均按照预后良好类型肾母细胞瘤方案给予放化疗,具体方案根据患儿临床分期分别为：Ⅰ期患儿采用长春新碱＋更生霉素6个月,Ⅱ期患儿采用长春新碱＋更生霉素15个月,Ⅲ期患儿采用长春新碱＋更生霉素＋阿霉素15个月（更生霉素与阿霉素间隔1.5个月）并辅以放疗,Ⅴ期患儿按照分期高的一侧放化疗.13例获得随访,随访时间6个月～15年,10例无瘤存活,3例死亡,1例失访.结论 FRN属于肾母细胞瘤的特殊类型,具有肿瘤体积大、双侧多见、侵袭性小、预后较好、对术前放化疗不敏感的临床特点,手术完整切除加术后化疗为主要治疗方式.

英文摘要：

Objective To explore the characteristic clinical profiles and treatment modalities of Fetal rhabdomyomatous nephroblastoma（FRN）.Methods A retrospective study was conducted for 14 FRN patients from Jan.2000 to Oct.2015.Their clinical data were collected including clinical presentations,pathology and treatment modalities.There were 8 males and 6 females with a mean age of 23 months.There were 3 cases at left side and right side 5 cases,bilateral 6 cases.2 patients were classified as stage Ⅰ,1 stage Ⅱ,5 stageⅢ and 6 stageⅤ.Abdominal mass was the main clinical presentation in 11 patients,and 1 case with hematuria,1 with abdominal pain,and 1 with vomit.Most tumors showed cysts or completely solid from the ultrasonography.Computed tomographic scan revealed a large inhomogeneous enhancement tumor from the kidney pole with necrotic,cystic,bleeding or calcification.Ultrasonography and Computed tomography （CT） had no different performance from Wilms＇ tumor.9 patients received preoperative chemotherapy,and the response was none in all of them.8 unilateral patients underwent tumor nephrectomy and another 4 had nephron-sparing surgery.Results Pathology showed that FRN contained more than 70% of fetal rhabdomyomatous tissue.Immunohistochemistry had no specificity,most FRN shows Desmin （＋） and Myogenin（＋）.Bilateral FRN tumors were seen in 2,one side with FRN and another side with nephroblastomatosis were seen in 3,one side with FRN and another side with Wilm＇s tumor was seen in 1 patient.Postoperative pathology confirmed FRN in all 14 cases.All patients received postoperative chemotheraphy：Act-D and VCR for 6 month（stage Ⅰ）,Act-D and VCR for 15 month（stage Ⅱ）,Act-D ＋VCR ＋ ADR and radiotherapy for 15 month（stageⅢ）.During follow-up of 6 months to 15 years,10 of them were alive without tumor and no evidence of recurrence.Conclusions FRN is a rare histologic variant of Wilm＇s tumor with less aggressive behavior.FRN usually has a huge volume and is bilateral with a poor respo