股骨近端骨原发性假肌源性血管内皮瘤一例影像及病理分析  

Imaging and pathological analysis of a case of primary pseudomyogenic hemangioendothelioma at the proximal Femur

作　　者：周兴顺 过哲 ZHOU Xing-shun;GUO Zhe(Department of Radiology,No.926 Hospital,Joint Logistics Support Force of PLA,Yunnan,661600,China;不详)

机构地区：[1]中国人民解放军联勤保障部队第九二六医院放射科,云南省661600 [2]首都医科大学附属北京积水潭医院放射科,北京100035

出　　处：《中国骨与关节杂志》2024年第8期627-629,共3页Chinese Journal of Bone and Joint

摘　　要：骨原发性假肌源性血管内皮瘤(pseudomyogenic hemangioendothelioma,PHE)是一种罕见的骨的中间型血管源性肿瘤,报道较少,且多以病理报道为主。现报告1例经手术与病理证实的骨原发性PHE的影像学表现并结合文献分析如下,以扩大该病的影像学资料库,助力对该病的影像诊断及鉴别诊断认识。Pseudomyogenic hemangioendothelioma(PHE)is a rare intermediate vascular tumor originating from bone,which has been less reported in radiological studies.It was previously known as epithelioid sarcomalike hemangioendothelioma(ES-HE).The tumor cells exhibit bright eosinophilic cytoplasm and morphologically resemble rhabdomyoblasts,while immunohistochemistry demonstrates endothelial differentiation.In the 2013 WHO classifcation of soft tissue and bone tumors,the term PHE was used.The tumor exhibits invasiveness,but metastasis is rare.It is classified as an intermediate vascular neoplasm.The 2020 classification maintains the same name and classification,and emphasizes the sensitivity of FOSB protein immunohistochemical staining in diagnosing PHE.Herein,we report the radiological fndings of a surgically and pathologically confrmed case of primary bone PHE.In this case,the lesion was localized in the proximal femur and exhibited irregular lytic bone destruction with partial sclerotic borders.Residual bony trabeculae was visible internally,and consistent with the literature and characteristics of intermediate-type PHE with no periosteal reaction or cortical breakthrough.CT and MRI enhancement scans revealed signifcant enhancement in line with its vascular origin.MRI also showed minimal focal hemorrhagic signals,and the MRI low signal margin around the lesion extended wider than the CT sclerotic margin,which were consistent with the histological features of focal bleeding and iron-hemosiderin deposition.

关 键 词：血管内皮瘤 股骨肿瘤 骨肿瘤 骨原发性假肌源性血管内皮瘤 

分 类 号：R739.9[医药卫生—肿瘤]