本文選題：腎腫瘤 + 先天性中胚層腎瘤　； 參考：《臨床與實(shí)驗(yàn)病理學(xué)雜志》2017年06期

【摘要】：目的探討先天性中胚層腎瘤(congenital mesoblastic nephroma,CMN)的臨床病理特征和分子遺傳學(xué)改變。方法回顧性分析9例CMN,總結(jié)其組織病理形態(tài)、免疫組化標(biāo)記及ETV6基因易位FISH檢測(cè)結(jié)果。結(jié)果 9例CMN均為2歲以內(nèi)的患兒,其中8例小于1歲。腫瘤平均直徑9.5 cm(3.2~15.0 cm)。經(jīng)典型3例,細(xì)胞型5例,混合型1例;其中5例可見囊性變,2例可見軟骨島。與經(jīng)典型相比,細(xì)胞型CMN的腫瘤直徑常更大,易出現(xiàn)出血、壞死,核分裂象更多見。免疫表型:腫瘤細(xì)胞表達(dá)vimentin,余未見特異性陽(yáng)性標(biāo)記,且均不表達(dá)WT-1。其中5例(4例細(xì)胞型和1例混合型)行ETV6基因易位FISH檢測(cè),3例細(xì)胞型CMN檢出ETV6基因易位,1例細(xì)胞型和1例混合型CMN未檢出ETV6基因易位。2例失訪,7例隨訪5~46個(gè)月,均未見復(fù)發(fā)。結(jié)論 CMN為少見的嬰幼兒腎臟腫瘤,具有獨(dú)特的臨床病理學(xué)特征,細(xì)胞型CMN有特異的ETV6基因易位。CMN多預(yù)后良好,需與其它兒童腎惡性腫瘤鑒別。
[Abstract]:Objective to investigate the clinicopathological features and molecular genetic changes of congenital mesodermal nephroma (CMN). Methods the histopathology, immunohistochemical staining and ETV6 gene translocation FISH were analyzed retrospectively in 9 cases of CMNs. Results all 9 cases of CMN were younger than 2 years old, 8 of them were less than 1 year old. The mean diameter of tumor was 9.5 cm(3.2~15.0 / cm ~ (-1). There were 3 cases of classical type, 5 cases of cellular type and 1 case of mixed type, of which 5 cases had cystic degeneration and 2 cases had cartilage island. Compared with the classical type, the tumor diameter of cellular CMN is much larger, it is prone to hemorrhage, necrosis and mitotic appearance. Immunophenotype: tumor cells expressed vimentin, but no specific positive markers were found, and none of them expressed WT-1. The ETV6 gene translocation FISH was detected in 5 cases (4 cell type and 1 mixed type). ETV6 gene translocation was detected in 3 cases of cellular CMN and 1 case of mixed type CMN without ETV6 gene translocation. All cases were followed up for 5 ~ 46 months and no recurrence was found. Conclusion CMN is a rare renal tumor in infants with unique clinicopathological features. Cellular CMN has a good prognosis with specific ETV6 gene translocation. It needs to be differentiated from other renal malignancies in children.
【作者單位】： 四川大學(xué)華西醫(yī)院病理科;
【基金】：國(guó)家自然科學(xué)基金(81272820、81272848、81572540)
【分類號(hào)】：R737.11

【參考文獻(xiàn)】

相關(guān)期刊論文 前3條

1 王晗;馬陽(yáng)陽(yáng);張大文;吳寶玉;房成;陳蓮;;先天性中胚層腎瘤的臨床病理分析[J];中華病理學(xué)雜志;2016年09期

2 鄒繼珍;何Pr;白云;蔡玲玲;肖萍;吳莎;李z魷，

本文編號(hào)：1882551