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

机译:先天性中胚层肾瘤:八例临床分析及文献复习

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摘要

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.
机译:先天性中胚层肾瘤(CMN)是一种间质性肾肿瘤。本研究的目的是回顾婴儿CMN的临床特征和结局。回顾性审查了2004年至2012年在复旦大学附属儿童医院治疗的8例CMN病例。已对所有8例患者进行了超声和计算机断层扫描。 2例实体瘤表现出与经典CMN一致的病理特征,5例表现出囊性,出血和坏死特征,钙化和病理与CMN的细胞变异相一致,1例表现出实体瘤,表现为病理特征与髓样CMN一致。组织学证实两名患者为经典CMN,六名患者为细胞CMN。对于外科手术,4例行了根治性肾切除术,1例行了半肾切除术,3例行了肿瘤摘除术。术前化疗2例,但无反应,术后化疗3例。两名患者失去随访,但其余六名患者存活至随访结束,无进一步并发症。平均随访时间为24.6个月。总之,CMN与Wilms肿瘤的鉴别诊断至关重要。成像特征与病理特征部分相关。手术是CMN的主要治疗方法,但术前化疗并不是特别有效。术后化疗的疗效尚待进一步研究,但预后良好。

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