Abstract

目的: 探讨原发于消化道CIC重排肉瘤的临床病理学特征。 方法: 回顾性分析2例原发于消化道的CIC重排肉瘤,包括病理学形态、免疫表型、遗传学特征及生存预后,并复习相关文献。 结果: 2例均为男性,年龄分别为44岁和54岁;肿瘤分别位于小肠和直肠,直径8.0 cm和9.0 cm。低倍镜下肿瘤呈分叶状或结节状,可见纤维性间隔和大片坏死。高倍镜下瘤细胞小至中等大,圆形或卵圆形,核形不规则,可见核分裂象,部分可见核仁。免疫组织化学显示2例均弥漫表达WT1,灶性表达CD99,Ki-67阳性指数分别为50%、70%。采用断裂分离探针荧光原位杂交(FISH)检测发现2例均有CIC基因断裂。2例分别于术后半个月、6个月发生远处转移,转移部位包括肺、腹腔及盆腔;1例于术后1个月死亡,1例存活。 结论: 发生在消化道的CIC重排肉瘤,组织病理学形态与尤因肉瘤较相似,免疫组织化学常表达WT1和CD99,确诊需借助FISH检测,患者预后较差。.

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