title: Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma: characterization of five cases.
abstract: OBJECTIVE
  To describe the imaging and histopathology of pseudomyogenic hemangioendothelioma.
  
  
  METHODS
  Five cases of pseudomyogenic hemangioendothelioma, which presented over the last 5 years, were retrieved from the files of the Royal National Orthopaedic Hospital. The imaging and histopathology were reviewed in all cases. Magnetic resonance imaging, which was available from all cases, was assessed for the following features: the number of lesions, location in soft tissue (superficial and or deep/subfascial) and bone, and the signal characteristics and morphology of individual lesions. Immunohistochemistry was performed in all cases to characterize the lesions.
  
  
  RESULTS
  Four of the five patients had multiple lesions involving a single limb. Bone was involved in 3 of the 5 individuals. All tumors diffusely expressed ERG and cytokeratins AE1/3, but not MNF116. CD31 was weakly positive in 4 cases. INI-1 expression was retained in all cases. Imaging features included ill-defined, infiltrative lesions in subcutaneous fat with extension to the adjacent skin, poor- to well-defined intramuscular nodules and predominantly intracortical focal bone lesions with rare medullary involvement.
  
  
  CONCLUSIONS
  Pseudomyogenic hemangioendothelioma represents a distinct recently characterized tumor type presenting in young adults, with a tendency towards multicentric bone and soft tissue involvement.
authors: Departments of Histopathology, Radiology and Surgery, Royal National Orthopaedic Hospital NHS trust, Brockley Hill, Stanmore, Middlesex HA7 4LP, UK.
authors: M Fernanda
authors: MF
authors: Amary
authors: Paul
authors: P
authors: O'Donnell
authors: Fitim
authors: F
authors: Berisha
authors: Roberto
authors: R
authors: Tirabosco
authors: Tim
authors: T
authors: Briggs
authors: Rob
authors: R
authors: Pollock
authors: Adrienne M
authors: AM
authors: Flanagan
mesh_terms: D000328:Adult; D001859:Bone Neoplasms / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D012983:Soft Tissue Neoplasms / Q000473:pathology*; D006113:United Kingdom; D055815:Young Adult
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