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[儿童肾原始神经外胚层肿瘤;其与肾母细胞瘤的鉴别诊断]

[Primitive neuroectodermal tumor of the kidney in children; its differential diagnosis with Wilms tumor].

作者信息

Popov S D, Sebire N J, Popova E D, Vujanic G M

出版信息

Arkh Patol. 2009 Nov-Dec;71(6):41-3.

Abstract

There may be a number of tumors made up by small round blue cells in the kidneys of children. One of them is primitive neuroectodermal tumor (PNET). The differences in therapeutic approaches determine the need to establish an accurate diagnosis. The differential diagnosis of PNET and the blastemal component of Wilms tumor can be difficult due to the similar histological pattern. There is a need for a close analysis of morphological manifestations, by keeping in mind the age of patients, and supplementary studies. A strong CD99 membrane expression and nuclear FLI1 expression in tumor cells are the signs of PNET. Reverse transcriptase-polymerase chain reaction and fluorescence in situ hybridization can determine PNET-specific translocations [t(11;22)(q24;q12), by involving the EWS gene.

摘要

儿童肾脏中可能存在多种由小圆形蓝细胞构成的肿瘤。其中之一是原始神经外胚层肿瘤(PNET)。治疗方法的差异决定了准确诊断的必要性。由于组织学模式相似,PNET与肾母细胞瘤的胚芽成分的鉴别诊断可能会很困难。需要密切分析形态学表现,同时牢记患者年龄,并进行补充研究。肿瘤细胞中强烈的CD99膜表达和核FLI1表达是PNET的特征。逆转录聚合酶链反应和荧光原位杂交可以确定PNET特异性易位[t(11;22)(q24;q12)],该易位涉及EWS基因。

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