13例动脉瘤样纤维组织细胞瘤的临床病理学特征

Clinicopathological features of 13 cases of aneurysmal fibrous histiocytoma

目的:分析动脉瘤样纤维组织细胞瘤(aneurysmal fibrous histiocytoma,AFH)的临床病理特征并有效进行鉴别诊断。方法:收集空军军医大学第一附属医院病理科2006年至2022年间初次诊断为真皮纤维瘤、皮肤神经鞘瘤、丛状神经纤维瘤、血管瘤样纤维组织细胞瘤、AFH的病例,由2名高年资软组织专科病理医师重新阅片复诊,最终确定诊断为AFH的患者共13例,对这13例进行回顾性分析。结果:在13例AFH患者中,男5例,女8例,年龄为19~43(中位数31.2)岁。镜下肿瘤细胞呈梭形,细胞质稀少,细胞无显著异型性,间质内含较多扩张的血管瘤样腔隙,并常见大量含铁血黄素沉着及多核巨细胞聚集,vimentin免疫组织化学染色阳性。结论:AFH较罕见,且极易误诊及漏诊,所以需与血管瘤样纤维组织细胞瘤等恶性肿瘤进行有效鉴别。

Objective: To analyze the clinicopathological features of aneurysmal fibrous histiocytoma(AFH) and to effectively make differential diagnosis.Methods: The cases of dermal fibroma, cutaneous schwannoma, plexus neurofibroma, hemangioma-like fibroblastoma, and AFH from 2006 to 2022 were collected from the Department of Pathology of First Affiliated Hospital of Air Force Medical University. A total of 13 patients diagnosed with AFH were finally confirmed, after reviewed by 2 senior soft tissue pathologists and retrospective analysis was performed on these 13 cases.Results: Among the 13 patients with AFH, 5 were males and 8 were females, aged 19-43(median 31.2) years. Microscopic tumor spindle cell cytoplasm is sparse, cells have no significant atypia, the interstitium contains more dilated hemangioma-like spaces, and a large number of hemosiderin-containing sedimentation and multinucleated giant cells are common, and the immunohistochemical stain results are positive for 13 cases of vimentin.Conclusion: AFH is rare and easy to be misdiagnosed, so it needs to be distinguished from malignant tumors such as hemangioma-like fibrohistoma.