Please confirm topic selection

Are you sure you want to trigger topic in your Anconeus AI algorithm?

Please confirm action

You are done for today with this topic.

Would you like to start learning session with this topic items scheduled for future?

Updated: Jun 21 2021

Hemangioendothelioma (hemangiosarcoma)

Images A_moved.jpg
  • 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
1 of 0
Private Note

Attach Treatment Poll
Treatment poll is required to gain more useful feedback from members.
Please enter Question Text
Please enter at least 2 unique options
Please enter at least 2 unique options
Please enter at least 2 unique options