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Intraosseous Angiosarcoma

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  • (Malignant hemangioendothelioma, hemangiosarcoma)
  • Solitary and multifocal malignant tumor of blood vessels occuring within a bone
    • Endothelial differentiation

IMPORTANCE

  • Rare lesion which because of contiguous and multicentricity of some lesions, require careful evaluation and awareness of neoplasm behavior
    • < 1% of primary bone malignancies

CLINICAL FEATURES

  • Bx can be very bloody!
  • M:F = 2:1
  • Most common in 3rd-5th decades, mean age ?50 ~50 yrs
  • Local pain and swelling may be longstanding

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  • Solitary, multifocal, similar in appearance to lytic metastases
    • Up to 1/3 with multifocal bone involvement
  • Cortical destruction with a soft tissue mass
    • Poorly marginated lucency of bone
  • May occur at the site of a bone infarct (MFH >> undifferentiated pleomorphic sarcoma >> osteosarcoma > fibrosarcoma > angiosarcoma in incidence), predominantly in M
  • Femur most common site
  • ± pathological fx

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  • Cavernous or in very aggressive lesions, a solid intramedullary mass, hemorrhagic, poorly defined margins
  • Typically 2-10 cm

HISTOLOGIC FEATURES

  • Complex anastomosing channels
  • Abnormalities in sizes and shapes of vascular channels
  • Abnormalities in organization of endothelial cells
  • Hobnail cells
  • Syncytial or papillary cell tufts
  • Shedding of individual and clusters of cells into the lumen of rudimentary vessels
  • Cellular atypia
    • Malignant spindled to epithelioid endothelial cells
  • Areas of solid sarcoma, loose fibroblastic stroma
  • Necrosis can be prominent
  • Immunohistochemistry
    • + factor VIII-related antigen
    • + CD-31 and CD-34 in vasoformative areas
    • + FLI-1
    • + keratin and EMA in epithelioid variant
      • Suspect intraosseous angiosarcoma if + keratin
  • EM
    • Weibel-Palade bodies specific for vascular endothelium: a single, membrane bound, rod-like body that contains parallel arrys arrays of microtubules on EM
  • "Angiotropism" is intravascular spread

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  • Metastatic disease and undifferentiated sarcoma
  • Invasive PVNS
  • Cellular hemangioma
    • Epithelioid hemangioma
  • Epithelioid hemangioendothelioma

DISEASE COURSE AND TREATMENT

  • Wide resection if lesion is solitary
  • Chemotherapy has been tried in high grade lesions
  • Amputation may be necessary in contiguous extremity lesions, XRT in contiguous axial lesions
  • Prognosis related to histologic grade
  • 5 yr survival when associated with a bone infarct: (14% in osteosarcoma, 22% in MFHundifferentiated pleomorphic sarcoma)

SPECIAL CONSIDERATIONS

 

  • Hemangioperictyoma

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