血管瘤样纤维组织细胞瘤的ALK表达 一种潜在的诊断陷阱
   --本文经《美国外科病理学杂志》授权发布,其他媒体转载或引用须经《美国外科病理学杂志》同意,否则追究法律责任。
  摘要:最近我们偶然遇到1例原发性肺血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma,AFH),因免疫组织化学(immunohistochemistry,IHC)显示间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)阳性,之前被误诊为炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)。为此,我们采用3种不同克隆号(D5F3、5A4、ALK1)的抗体对11例AFH、15例IMT和11例滤泡树突细胞(follicular dendritic cell,FDC)肉瘤的ALK表达进行检测。使用ALK断裂双色荧光探针进行荧光原位杂交(fluorescence  insitu hybridization,FISH)对ALKIHC阳性病例进行分析。结果显示,大多数AFH病例至少表现一种克隆号(9/11的D5F3阳性,6/9的5A4阳性,1/9的ALK1阳性)的ALKIHC阳性,呈胞浆中等至强阳性染色。通过使用ALK基因拷贝数在1.6~2.1间的FISH检测发现,ALKIHC阳性的AFH病例均无ALK基因重排(0/8)。IMT中67%的病例ALKIHC阳性(10/15的D5F3阳性,8/15的5A4阳性,7/15的ALK1阳性),FISH显示,10例病例中有9例的ALK基因重排阳性。所有FDC肉瘤的ALKIHC(D5F3和5A4)均为阴性。我们的结果显示,AFH中常见ALK的表达,特别在使用D5F3和5A4高敏性抗体和增敏检测系统,也可能是导致AFH与IMT诊断混淆的潜在原因。AFH中ALK表达的具体分子机制尚不清楚,并非为ALK基因重排或基因扩增所致。
  关键词:间变性淋巴瘤激酶(ALK),血管瘤样纤维组织细胞瘤(AFH),炎性肌纤维母细胞瘤(IMT),上皮样纤维组织细胞瘤(EFH),原发性肺黏液样肉瘤(PPMS)
  (Am J Surg Pathol 2019;43:93-101)
  美国外科病理学杂志中文版2019年第三期摘要NO.10
  ( 时雨 张华蓉 翻译 阎晓初 审校 )








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