小脑HGNET-BCOR肿瘤—3例小脑HGNET-BCOR肿瘤的临床病理与分子特征

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  ——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。

  具有BCOR的中枢神经系统( central nervous system,CNS )高级神经上皮肿瘤 ( high-grade neuroepithelial tumor,HGNET)(CNS HGNET-BCOR)是最近描述的一种分子实体。我们报告3例具有相同临床表现和病理特点的CNS HGNET-BCOR新病例。3例均为儿童病例,年龄3~7岁,表现出小脑巨大占位性病变。病理学特征包括一致的、具有纤细染色质的梭形到卵圆形细胞的增生,与丰富的树枝状毛细血管网。甲基化分析将这些病例归入CNS HGNET-BCOR肿瘤。聚合酶链式反应分析证实,这3例病例存在BCOR羧基端内部串联重复(internal tandem duplications in the C-terminus of BCOR,BCOR-ITD),就是这些肿瘤的特征。免疫组化染色显示核BCOR强表达。其中2例在手术后6个月内局部复发,第3例在手术完全切除后接受全脑全脊髓放疗,随后接受伊立替康、替莫唑胺和伊曲康唑节拍式化疗,在诊断14个月后仍保持无病状态。总的来说,CNS HGNET-BCOR是一种罕见的、发生于年轻患者预后较差的肿瘤。BCOR核表达高度提示BCOR-ITD。CNS HGNET-BCOR 应归入“ 胚胎性肿瘤 ”类别还是“间叶性、非脑膜上皮性肿瘤”有待于进一步阐明。由于CNS HGNET-BCOR 与肾透明细胞肉瘤享有相同的病理学特征和特征性的BCOR-ITD,因此,这些肿瘤可能是相同实体不同部位的亚型。
  Am J Surg Pathol 2017;41:1254–1260
美国外科病理学杂志中文版2018年第一期摘要No.5
  (张声 翻译/审校)
  The American Journal of Surgical Pathology中文版声明:
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