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目的 探讨皮下原发淋巴瘤样肉芽肿(lymphomatoid granulomatosis,LYG)的临床病理特征.方法 观察1例皮下原发LYG的临床、组织学、免疫表型及分子病理学特征,并复习相关文献.结果 患者男性,78岁,无意间发现右侧腋窝肿块10余天.肿物界尚清,似有包膜,切面灰黄、灰红色,质地中等.镜下肿物中央见大量凝固性坏死,周边区域呈脂膜炎形态改变,伴多形性淋巴细胞样细胞浸润,可见血管中心性和血管破坏性浸润,病理性核分裂象偶见.免疫表型:非典型大淋巴样细胞CD45RB、CD20、CD30均阳性,CD3、CD15、CD56、TIA-1、Granzyme B、ALK、CD21、Langerin、CD1a、S-100和CK(AE1/AE3)均阴性,Ki-67增殖指数50％～60％.EBER原位杂交检测显示阳性细胞>20/HPF.抗酸及TB-DNA检测均不支持结核.分子病理:IgH+IgK基因重排显示存在IgK单克隆性增生;TCRB+TCRG基因重排显示不存在单克隆性增生的T细胞群.结论 肺外皮下原发LYG是一种极罕见的肿瘤,经形态学、免疫组化标记与分子病理学检测相结合可明确诊断.“,”Purpose To investigate the clinicopathological features of primary subcutaneous lymphomatoid granulomatosis (LYG). Methods A case of primary subcutaneous LYG was observed by analysis of the clinical, histological features, immunophenotype and molecular pathology with review of the related literature. Results The male patient, 78-year-old, inadvertently found a mass of right axillary for more than 10 days. The boundary of the mass was clear, it seemed to have a capsule, the cut surface was grayish yellow and grayish red, the texture was medium. A large amount of coagulative necrosis was observed in the center of the mass under microscope. The peripheral area showed a morphological change of panniculitis, accompanied by pleomorphic lymphoid infiltration, showed central and vascular destructive infiltration, pathological mitosis was occasionally observed. Immunophenotyping showed that atypical large lymphoid cells expressed CD45 RB, CD20, CD30, while CD3, CD15, CD56, TIA-1, Granzyme B, ALK, CD21, Langerin and CD1 a, S-100 and CK (AE1/AE3) were negative. The proliferation index of Ki-67 ranged from 50％ to 60％. EBER in situ hybridization showed that positive cells were> 20/HPF.Neither acid fast staining nor TB-DNA testing supported tuberculosis. Molecular pathology found clonal Ig K gene rearrangement, TCRB + TCRG gene rearrangement showed the absence of monoclonal proliferating T cell population. Conclusion The primary subcutaneous LYG is a rare tumor. which can be diagnosed by combination of morphology, immunophenotype and molecular pathology.