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《Radiologic Practice》 2018-04
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Imaging and clinicopathological characteristics of primary skeletal pseudomyogenic(epithelioid sareoma-like)haemangioendothelioma

YI Li-lei;LIU Zhuang-sheng;XIE Le;Department of Radiology,the Affiliated Foshan Hospital of Traditional Chinese Medicine,Guangzhou University of Traditional Chinese Medicine;  
Objective:To explore the clinical and imaging characteristics of pseudomyogenic(epithelioid sareoma-like)haemangioendothelioma(PHE/ES-HE)of bone.Methods:The clinical data,Xray,CT and MR images and histopathological appearances of 6 cases with primary skeletal PHE/ESHE were retrospectively studied,related literature was reviewed,and the imaging and clinicopathological features were summarized.Results:There were 4 males and 2 females with an average age of 31.8 years,pain at the involved area lasted 3 months to 5 years.There were 31 skeletal lesions which involved right ilium and bones of right lower extremity in 6 cases.There were solitary lesions in 3 cases and multifocal lesions in other 3 cases.All of the lesions showed a well-defined osteolysis and marked enhancement after injecting contrast agents.Three solitary lesions demonstrated heterogeneous CT density and MRI intensity with sclerotic rim,cortical defects and large soft tissue mass.Residual bone trabecula and cystic degeneration were present in these 3 lesions,and calcification in one lesion.Among the 28 multifocal lesions,19 lesions showed homogeneous CT density and MRI intensity,15 lesions showed sclerotic rim,and no lesion contained residual bone,cystic degeneration or calcification.There was misdiagnosis of imaging examination in all cases.Among them,2 cases were misdiagnosed as multiple myeloma,2 cases were misdiagnosed as giant cell tumor of bone,one case was misdiagnosed as fibrous dysplasia of bone,and the other one was misdiagnosed as epithelioid hemangioendothelioma of bone.The proliferation of oval cells or circular epithelial-like cells and fusiform cells were seen under the microscope,no vascular lacuna was found.In only one case,the formation of vacuoles was found in the cytoplasm.Some inflammatory cells were scattered in the interstitial tissue.Immunohistochemical staining showed FLi-1(+),CD31(+)partly,FⅧα(+)partly,AE1/AE3(+)partly,Ki-67(+)20% and CD34(-).Conclusion:Although there are some specific imaging and clinicopathological features,primary PHE/ES-HE of bone is still easy to be misdiagnosed before surgery.The differential diagnosis should be made from multiple myeloma and giant cell tumor of bone.
【Fund】: 佛山市重点专科培育项目资助基金(Fspy3-2015019)
【CateGory Index】: R445.2;R730.44;R738.1
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