title: [Primary intermediate hemangioendothelioma of bone: a study of 5 cases].
abstract: OBJECTIVE
  To study the radiologic and pathologic features of primary intermediate hemangioendothelioma of the bone.
  
  
  METHODS
  Five cases of primary intermediate hemangioendothelioma of bone encountered in the past three years were enrolled into the study. The clinical, radiologic, pathologic and immunohistochemical features of the tumors were reviewed.
  
  
  RESULTS
  The patients included 3 children with Kaposiform hemangioendothelioma and 2 elderly with retiform hemangioendothelioma. Four of the cases affected long bones and the remaining case affected the clavicle. One case showed multifocal involvement of the humerus. Radiologically, the tumors showed borderline to low-grade bony destruction, with various degrees of cortical defect. Intralesional or perilesional bone formation was demonstrated in 4 cases and radial spicules were seen in 1 case. The histopathologic features of primary intermediate hemangioendothelioma of bone were similar to those of soft tissue, except for the presence of reactive bone formation. Immunohistochemically, the tumor cells were positive for CD31 (5/5), CD34 (5/5), vimentin (5/5) and smooth muscle actin (3/5) but negative for cytokeratin and epithelial membrane antigen.
  
  
  CONCLUSIONS
  Primary intermediate hemangioendothelioma of bone is a distinct entity and similar histologic classification applies as in its soft tissue counterparts. Comparison of the biologic behavior requires long-term follow-up studies.
authors.affiliation: Department of Pathology, Shanghai Sixth People's Hospital, Shanghai Jiao Tong University, Shanghai 200233, China.
authors.forename: Hui-zhen
authors.initials: HZ
authors.lastname: Zhang
authors.forename: Zhi-ming
authors.initials: ZM
authors.lastname: Jiang
authors.forename: Jun
authors.initials: J
authors.lastname: Zhou
authors.forename: Shi-xun
authors.initials: SX
authors.lastname: Yang
authors.forename: Qin-he
authors.initials: QH
authors.lastname: Fan
authors.forename: Yun-shan
authors.initials: YS
authors.lastname: Tan
authors.forename: Xue-li
authors.initials: XL
authors.lastname: Wang
mesh_terms: D000199:Actins / Q000378:metabolism; D018952:Antigens, CD34 / Q000378:metabolism; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D002648:Child; D002968:Clavicle / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D005269:Femur / Q000473:pathology; D006390:Hemangioendothelioma / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D006394:Hemangiosarcoma / Q000473:pathology; D006801:Humans; D006811:Humerus / Q000473:pathology; D007150:Immunohistochemistry; D007223:Infant; D059885:Kasabach-Merritt Syndrome / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000378:metabolism; D011859:Radiography; D012514:Sarcoma, Kaposi / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D014746:Vimentin / Q000378:metabolism
chemical_list: C541116:ACTA2 protein, human; D000199:Actins; D018952:Antigens, CD34; D019408:Platelet Endothelial Cell Adhesion Molecule-1; D014746:Vimentin