Updated: 10/6/2016

Hemangioendothelioma (hemangiosarcoma)

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Introduction
  • Hemangioendothelioma (or epitheliod hemangioendothelioma in soft tissue) is considered a rare sarcoma showing endothelial differentiation
    • distinct histologically and molecularly from benign hemangioma and high-grade angiosarcoma
  • Epidemiology
    • demographics
      • occurs most commonly in 4th - 5th decade 
      • women affected more than men
    • location
      • more commonly in soft-tissue > bone 
      • usually occurs in the lower extremity
    • risk factors
      • exposure to arsenic 
  • Pathophysiology
    • pathoanatomy
      • arise from the lining of blood vessels
    • cellular biology
      • stratifed into two groups based on size of tumor and mitotic activity
        • classic 
        • 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
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 diagnosis
  • 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)
Ibank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A tibia    
Case B finger    
Case C tibia
 
Case D tibia
   
(1) - histology does not always correspond to clinical case 
 

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