本文選題：EWSR基因 + 肺原發(fā)性黏液樣肉瘤　； 參考：《中國(guó)癌癥雜志》2017年05期

【摘要】：背景與目的:肺原發(fā)性黏液樣肉瘤是一種非常罕見的軟組織腫瘤。最近有學(xué)者發(fā)現(xiàn)該腫瘤具有特異性的EWSR1基因易位。該研究旨在探討伴EWSR1基因易位的肺原發(fā)性黏液樣肉瘤的臨床病理學(xué)特征及其鑒別診斷。方法:回顧性分析復(fù)旦大學(xué)附屬腫瘤醫(yī)院病理科診斷的6例伴EWSR1基因易位的肺原發(fā)性黏液樣肉瘤,收集臨床及影像學(xué)資料及組織病理學(xué)形態(tài),采用免疫組織化學(xué)法分析免疫學(xué)表型,采用熒光原位雜交(fluorescence in situ hybridization,FISH)檢測(cè)EWSR1基因融合狀態(tài),并復(fù)習(xí)相關(guān)文獻(xiàn)。結(jié)果:患者均為成年人,其中男性4例,女性2例,發(fā)病年齡23~64歲,中位年齡44歲。大體上,腫瘤大小2.0~5.5 cm,腫瘤境界較清楚,切面質(zhì)韌,灰白灰黃色,膠凍樣。鏡下觀察,所有病例均與支氣管關(guān)系緊密,腫瘤細(xì)胞主要由梭形細(xì)胞或多邊形細(xì)胞組成,排列呈條索狀、梁狀或網(wǎng)狀結(jié)構(gòu),背景為多少不等的黏液樣基質(zhì)。該腫瘤缺乏特異性標(biāo)志物,但腫瘤細(xì)胞可不同程度表達(dá)上皮膜抗原(epithelial membrane antigen,EMA)。FISH檢測(cè)結(jié)果顯示EWSR1基因重排陽(yáng)性。隨訪4~29個(gè)月,其中5例無(wú)瘤生存,1例出現(xiàn)胸膜及骨轉(zhuǎn)移。結(jié)論:伴有EWSR1基因重排的肺原發(fā)性黏液樣肉瘤是一種極為罕見的低度惡性的肉瘤。組織學(xué)上有一定的特征性改變,熟悉其瘤譜及基因?qū)W特征有助于診斷和鑒別診斷。
[Abstract]:Background & objective: primary myxoid sarcoma of the lung is a rare soft tissue tumor. Recently, some researchers have found that the tumor has specific translocation of EWSR1 gene. The aim of this study was to investigate the clinicopathological features and differential diagnosis of primary myxoid sarcoma with EWSR1 gene translocation. Methods: six cases of pulmonary primary myxoid sarcoma with EWSR1 gene translocation were analyzed retrospectively. Clinical and imaging data and histopathology were collected. Immunohistochemistry was used to analyze the immunophenotype, fluorescence in situ hybridization in situ hybridization (fish) was used to detect the fusion status of EWSR1 gene, and the related literatures were reviewed. Results: all the patients were adults, including 4 males and 2 females. The onset age was 2364 years old and the median age was 44 years old. In general, the tumor size is 2.0 ~ 5.5 cm, the boundary of tumor is clear, the cut surface is tough, grayish gray and yellow, gelatinous. The tumor cells were mainly composed of fusiform cells or polygonal cells, arranged in a stripe, beamlike or reticular structure, and the background was of mucoid matrix. There was no specific marker in the tumor, but the epithelial membrane antigen epithelial membrane antigen-EMA-EMA-.FISH showed that the EWSR1 gene rearrangement was positive in different degree. Follow-up for 4 ~ 29 months showed that 5 cases survived without tumor and 1 case had pleural and bone metastasis. Conclusion: primary myxoid sarcoma with EWSR1 gene rearrangement is a rare low grade malignant sarcoma. There are some characteristic changes in histology. It is helpful for diagnosis and differential diagnosis to be familiar with the tumor spectrum and genetic characteristics.
【作者單位】： 復(fù)旦大學(xué)附屬腫瘤醫(yī)院病理科 復(fù)旦大學(xué)上海醫(yī)學(xué)院腫瘤學(xué)系;復(fù)旦大學(xué)附屬腫瘤醫(yī)院胸外科 復(fù)旦大學(xué)上海醫(yī)學(xué)院腫瘤學(xué)系;
【基金】：國(guó)家自然科學(xué)基金面上項(xiàng)目(81472173)
【分類號(hào)】：R734.2

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本文編號(hào)：1856480