骨原发上皮样血管肉瘤临床病理学分析

摘要

目的：探讨骨原发上皮样血管肉瘤（ PEAB）的临床病理特点。方法报道1例骨原发上皮样血管肉瘤临床资料、组织学及免疫组化染色结果及随访结果。结果该例骨原发上皮样血管肉瘤的临床症状为局部疼痛，查体发现局部压痛，镜下示上皮样肿瘤细胞排列成假腺样、乳头状，可见原始血管结构形成。免疫组化显示瘤细胞高表达CD31、CKpan、Vimentin，并表达CD34，而EMA、SMA、S-100、Myogenin及HMB45等均阴性。结论骨的原发上皮样血管肉瘤罕见，其组织结构极易被误诊为转移癌，诊断需结合临床及影像学资料，对组织病理学的详细观察及免疫组化染色的协助，对于正确诊断和鉴别诊断有重要意义。%Objective To investigate the clinicopathological characteristics,diagnosis and differential diagnosis,treatment and prognosis of primary epithelioid angiosarcoma in bone PEAB.Methods One case of PEAB was retrospectively reviewed on the clinical data,his-tological and immunohistochemical features,the follow-up,and the related literature.Results The symptom of the case was localized pain.Physical examination results showed that there was local tenderness.Histologically,PEAB was comprised of the epithelioid-like cells arranged in pseudoglandular or papillary patterns.Structure of primitive vascular can be seen in some areas.Immunohistochemical-ly,the tumor cells were stained positive for CD31,CK,vimentin and CD34,negative for SMA,S-100,EMA,Myogenin and HMB45.Con-clusions PEAB is very rare.Because of its histopathological character,it is likely to be misdiagnosed as metastatic carcinoma.An ac-curate diagnosis is based on clinical manifestations,imaging,histopathological test and immunohistochemical staining.