纵隔滑膜肉瘤 21例经分子检测证实病例的临床病理分析
  ——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。

  滑膜肉瘤(synovial sarcoma,SS)是一种以SS18-SSX1/2融合为特征的易位相关肉瘤,最常发生于年轻成人四肢。尽管SS发生于胸膜肺实质相对常见,但纵隔却是相当罕见的原发部位;对此,现有的文献多为个案或小宗病例报道,且多未经分子检测证实。纵隔SS病例选自本机构病例及会诊资料。所有病例均经荧光原位杂交检测SS18(n=6)或逆转录聚合酶链反应检测SS18-SSXl/2(n=15)。21例患者发生纵隔SS(15例男性;平均年龄,38岁;范围,21~75岁)。仅有1例患者年龄超过50岁。肿瘤平均大小为13.5cm(范围:6.4~23cm)。1例肿瘤为双相型,其余为单相型,11例为低分化型(52%)。10例具有已知的融合转录基因,其中6例为SS18-SSX2,4例为SS18-SSX1。16例有随访资料(平均:18.9个月;范围:5~45个月):14例具有局部进展或复发,6例有转移。随访时间5~32个月期间,16例患者中有11例(69%)因病死亡,5例患者在最后随访(6~45个月)时带病生存。纵隔SS是一种非常罕见的侵袭性恶性肿瘤,最常发生于50岁以下患者。大多为单相型且生长到相当大体积时才被发现。低分化形态学常见。SS应纳入表现为梭形细胞、双相型及低分化纵隔肿瘤的鉴别诊断。鉴于纵隔为SS的罕见发生部位,推荐进行分子检测加以证实。
  关键词:滑膜肉瘤,纵隔,胸部
Am J Surg Pathol 2018;42:761-766
美国外科病理学杂志中文版2019年第1期全文No.1
任俊奇 翻译 朱朋成 审校






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