摘要目的:探讨肝脏血管肉瘤的临床病理特点，并评估α地中海贫血伴智力低下综合征X连锁(Alpha-thalassemia-mental rebardation X-linked，ATRX)免疫组化染色在其诊断中的应用。方法:回顾性分析北京医院病理科2014年至2023年诊断的7例肝脏血管肉瘤患者的病理及临床资料，并采用免疫组化方法检测ATRX表达情况。结果:患者中6例为体检时偶然发现，1例因腹痛就诊。6例患者为多发结节，仅有1例为单发结节。镜下形态多样，高分化血管肉瘤可见肝板萎缩，肝窦内衬覆肿瘤细胞，低分化血管肉瘤细胞异型性较大，需要与其他肉瘤相鉴别。肿瘤细胞均表达血管内皮标记血小板内皮细胞黏附分子-1(platelet endothelial cell adhesion molecule，CD31)、造血祖细胞抗原(CD34)及Ets相关基因(Ets related-gene，ERG)等，并有4例中-低分化血管肉瘤肿瘤细胞出现ATRX表达缺失。结论:肝血管肉瘤表现及影像学检查无特异性，综合分析患者的临床及形态学，并辅以血管标记及ATRX免疫组化染色，有助于正确的病理诊断。

abstractsObjective:To investigate the clinicopathological characteristics and the application of ATRX(alpha-thalassemia mental retardation X-linked)in hepatic angiosarcoma.Methods:We retrospectively analyzed the pathological and clinical data of seven patients diagnosed with hepatic angiosarcoma at the Department of Pathology, Beijing Hospital, between 2014 and 2023.ATRX immunohistochemical staining was conducted.Results:Among the patients, six were identified incidentally during physical examinations, while only one patient presented with abdominal pain.Six patients had multiple nodules, whereas one had a solitary tumor.The morphological features were diverse; high-grade angiosarcoma exhibited liver plate atrophy and hepatic sinusoids lined with atypical cells, while low-differentiated angiosarcoma displayed a higher degree of atypia and should be differentiated from other sarcomas.Tumor cells expressed vascular endothelial markers, including CD31(platelet endothelial cell adhesion molecule-1), CD34(hematopoietic progenitor cell antigen CD34), and ERG(Ets-related gene).Additionally, loss of ATRX expression was observed in four patients.Conclusions:Hepatic angiosarcoma is characterized by a lack of specific clinical manifestations and imaging findings.A comprehensive analysis of clinical symptoms, morphological features, and immunohistochemical staining—particularly for vascular markers and ATRX—is essential for an accurate pathological diagnosis.