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先天性中胚层肾瘤:8例临床分析及文献复习

Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature.

作者信息

Wang Zuo-Peng, Li Kai, Dong Kui-Ran, Xiao Xian-Min, Zheng Shan

机构信息

Department of Pediatric Surgery, Children's Hospital of Fudan University, Shanghai 201102, P.R. China.

出版信息

Oncol Lett. 2014 Nov;8(5):2007-2011. doi: 10.3892/ol.2014.2489. Epub 2014 Sep 1.

Abstract

Congenital mesoblastic nephroma (CMN) is a mesenchymal renal tumor. The aim of the present study was to review the clinical characteristics and outcome of CMN in infants. A retrospective file review was conducted of eight cases of CMN treated at the Children's Hospital of Fudan University between 2004 and 2012. Ultrasound and computerized tomography scans had been performed on all eight patients. Two cases presented with a solid tumor and exhibited pathological features consistent with those of classic CMN, five cases exhibited cystic, hemorrhagic and necrotic characteristics, with calcification and pathology consistent with the cellular variant of CMN and one case presented with a solid tumor, which exhibited pathological features consistent with ceullular CMN. Histology confirmed classic CMN in two patients and cellular CMN in six patients. For surgical intervention, four cases had radical nephrectomy, one case had a half nephrectomy and three cases had tumor enucleation performed. Two cases had received pre-operative chemotherapy, but exhibited no response, and three cases received post-operative chemotherapy. Two patients were lost to follow-up, but the remaining six patients survived to the end of follow-up without further complications. The mean follow-up time was 24.6 months. In conclusion, the differential diagnosis between CMN and Wilms' tumor is critical. Imaging characteristics are partially correlated with pathological characteristics. Surgery is the main treatment for CMN, but pre-operative chemotherapy is not particularly effective. The efficacy of post-operative chemotherapy requires further investigation, but the prognosis is positive.

摘要

先天性中胚层肾瘤(CMN)是一种间叶性肾肿瘤。本研究的目的是回顾婴儿CMN的临床特征及预后。对2004年至2012年在复旦大学附属儿科医院接受治疗的8例CMN病例进行了回顾性病历分析。所有8例患者均进行了超声和计算机断层扫描。2例表现为实性肿瘤,病理特征与经典CMN一致;5例表现为囊性、出血性和坏死性特征,伴有钙化,病理与CMN细胞型一致;1例表现为实性肿瘤,病理特征与细胞型CMN一致。组织学检查证实2例为经典CMN,6例为细胞型CMN。手术干预方面,4例行根治性肾切除术,1例行半肾切除术,3例行肿瘤剜除术。2例接受术前化疗,但无反应,3例接受术后化疗。2例失访,但其余6例随访结束时均存活且无进一步并发症。平均随访时间为24.6个月。总之,CMN与肾母细胞瘤之间的鉴别诊断至关重要。影像学特征与病理特征部分相关。手术是CMN的主要治疗方法,但术前化疗效果不佳。术后化疗的疗效有待进一步研究,但预后良好。

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