Purpose To investigate the clinical-pathological characteristics of the primary thyroid intravascular large B-cell lymphoma (PT-IVLBCL). Methods The clinical-patho-logical data of the PT-IVLBCL were analyzed retrospective. A comprehensive analysis of the literature on IVLBCL published between 1991-2017 in China were performed. Results A thy-roid mass was identified in a physical examination of a 68-year-old male who initially presented with dyspnea accompanied by intermittent headache for about 1 month. Pathological results showed that large atypical lymphoma-like cells filled the small vessel capillaries in the lesion area. Immunohistochemical staining revealed that the lymphoma-like cells were positive for CD20, Pax-5, BCL-2 and MUM1. The Ki-67 index was estimated to be approximately 85％. Conclusion IVLBCL is a rare and aggressive disease that is easy to be misdiagnosed or missed, because it's clinical presentations are non-specific. The correct diagnosis depends on pathology. IVLBCL is known for its rapid progression and poor prognosis, but timely diagnosis and treatment with chemotherapy can improve patients survival.%目的 探讨甲状腺血管内大B细胞淋巴瘤(intravascular large B-cell lymphoma, IVLBCL)的临床病理学特征.方法 分析1例甲状腺IVLBCL的临床病理特征,并系统性复习我国1991 ～2017年文献报道的IVLBCL.结果 甲状腺肿块病检示病损区小血管腔扩张,并充满大异型淋巴样细胞.免疫表型:淋巴样细胞CD20、Pax-5、BCL-2和MUM1均阳性,增殖指数85％.结论 IVLBCL临床较罕见,无特殊性表现,易误、漏诊,明确诊断依靠病理检查.IVLBCL预后较差,早期诊断并及时接受有效化疗可延长患者生存期.
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