Updated: 6/21/2021

Hemangioendothelioma (hemangiosarcoma)

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  • summary
    • Hemangioendothelioma, also known as hemangiosarcoma, is a rare, malignant, vascular tumor with endothelial differentiation that is more common in soft tissues than bone. The tumor typically presents in patients between ages 30 and 40 with regional pain.
    • Diagnosis is made with biopsy and histology showing angiocentric growth expanding the vessel wall with surrounding sclerosis. 
    • Treatment is usually radiotherapy alone for low-grade lesions. Surgical resection with or without radiation is indicated for high-grade lesions. 
  • Epidemiology
    • Demographics
      • occurs most commonly in 4th - 5th decade
      • women affected more than men
    • Anatomic location
      • more commonly in soft-tissue > bone
      • usually occurs in the lower extremity
    • Risk factors
      • exposure to arsenic
  • Etiology
    • Pathophysiology
      • pathoanatomy
        • arise from the lining of blood vessels
      • cellular biology
        • distinct histologically and molecularly from benign hemangioma and high-grade angiosarcoma
        • stratified into two groups based on size of tumor and mitotic activity
          • classic
          • malignant
  • Presentation
    • Symptoms
      • local pain
      • not usually associated with swelling
  • Imaging
    • Radiographs
      • recommended views
        • 2 or 3 views of affected bone
      • findings
        • lytic lesion with no reactive bone
        • areas of cortical destruction and cortical expansion are common
    • CT
      • indication
        • cross-sectional imaging to better define the area of concern
      • findings
        • multifocal eccentric lytic lesions in bone
        • matrix mineralization may be present
        • calcification and periosteal reaction is rare
    • MRI
      • indication
        • cross-sectional imaging to better define the area of concern
      • findings
        • high signal intensity on T2-weighted images
        • edematous bone reaction surrounding the lesion
  • Histology
    • Macroscopic
      • angiocentric growth expanding the vessel wall
      • obliterating the lumen and spreading centrifugally into surrounding tissue
      • induce a sclerotic response
    • Microscopic
      • arranged in single files, cords and small nests
      • typically lack well-formed vascular channels, with only immature, intracytoplasmic lumina
  • Differential
    • Langerhans cell histiocytosis (LCH), angiosarcoma, infection, myeloma, metastasis, and lymphoma
      • the diagnosis of hemangioednthelioma requires histopathologic analysis
  • Treatment
    • Nonoperative
      • radiation therapy alone
        • indications
          • low grade lesion (classic)
      • chemotherapy
        • the role of chemotherapy is not yet clear
    • Operative
      • surgical resection +/- radiotherapy
        • indications
          • high grade lesion (malignant)
  • Prognosis
    • Natural history of disease
      • malignant
        • tumor > 3cm and >3 mitoses/50 HPFs have 5 year survival of 59%
      • classic
        • 100% survival if lack these features
    • Prognostic features
      • 20 - 30% of tumors metastasize
      • visceral involvement is considered the most important criterion in predicting survival
Evidence (2)
EXPERT COMMENTS (5)
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