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-  2018 

原发性气管或肺恶性血管球瘤的临床特点

DOI: doi:10.7507/1671-6205.201710018

Keywords: 原发性, 气管,, 恶性血管球瘤

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Abstract:

目的探讨原发性气管或肺恶性血管球瘤(MGT)的临床特点。 方法通过报道本院 1 例原发性气管 MGT,并检索国内外数据库检索已发表的原发性气管或肺 MGT 的案例,对其临床表现、影像学表现、气管镜表现、病理学表现尤其是免疫组织化学特点、诊断及鉴别诊断、治疗和预后进行归纳总结。 结果患者男性,咳嗽、胸闷、气促、呼吸困难。气管内 MGT,气管镜下射频消融+冷冻治疗,患者拒绝化疗,双肺转移,术后 2 个月死亡。检索到 16 篇文献共 17 例原发性气管或肺 MGT,其中 5 例原发于气管,12 例原发于肺。其临床表现、影像学表现、气管镜表现无特异性,诊断主要依靠病理学尤其是免疫组织化学染色结果。气管 MGT 的治疗首先考虑外科手术节段切除,其预后尚可。肺 MGT 的治疗有肺叶切除术或气道介入治疗,其预后均较差。目前原发于气管或肺 MGT 的治疗,需早期治疗,由于气道介入及化疗的疗效不确切,治疗仍需首先考虑外科手术切除。 结论原发性气管或肺 MGT 为罕见病,其临床表现、实验室检查及影像学无特征性表现,极易漏诊、误诊,最终需依靠活检进行病理学分析尤其是免疫组织化学染色分析以明确诊断,其治疗方法仍有待进一步探讨

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