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Intraosseous Angiosarcoma


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


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


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


  • 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 (undifferentiated pleomorphic sarcoma >> osteosarcoma > fibrosarcoma > angiosarcoma in incidence), predominantly in M
  • Femur most common site
  • ± pathological fx


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


  • 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 arrays of microtubules on EM
  • "Angiotropism" is intravascular spread


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


  • 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 undifferentiated pleomorphic sarcoma)



  • Hemangioperictyoma

    • Composed of the pericytes of Zimmerman, occurring in adults, especially the thigh, pelvis, and retroperitoneum, and may be either benign or malignant, occurring in bone or soft tissue
  • Infantile Hemangiopericytoma

    • Have a better prognosis than the adult form
    • Ulceration and bleeding from the mass may be life-threatening
  • Hemangioendothelioma

    • (grade I and II; low-grade angiosarcoma)
    • Mean age is 35-40, presenting with a dull aching pain
    • Has been reported 30 yrs after IF of a tibial fx
    • Spinal lesions have been txd w/ XRT alone, curettage and XRT, anterior resection alone, and anterior resection and XRT (numbers too small to make recommendations, although the one pt txd w/ XRT alone developed pulmonary metastasis
    • Stabilization is recommended to diminish chronic back pain
    • Differentiate from epithelioid hemangioma of bone

    • 2 reported cases
    • Spindle cell proliferation with vascular spaces and papillary tufts of hobnail endothelial cells
    • Wide resection and aggressive curettage have been reported


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