Angiosarcoma

Angiosarcoma 

Updated: 11/18/2020

© Jun Wang, MD, PhD

General features
  • Malignant neoplasm arising from endothelial cells
  • More common in adults 
  • Very rare in children
  • High grade
  • Rapid growing, aggressive
  • Metastasis may be seen in lymph node, unlike other sarcomas
Risk factors
  • Chronic lymphedema
  • Radiation
  • PVC
  • HIV
  • Environmental carcinogens
Clinical presentations
Breast: 3-12 years after RADIATION therapy for carcinoma
Extremities: Associated with lymphedema ~ 10 years after radical mastectomy for breast cancer, arising from dilated lymphatics, not associated with radiation therapy (lymphangiosarcoma)
Liver: Associated with arsenic, Thorotrast, polyvinyl chloride, radiation to other sites, introduction of foreign material
Skin: If related to chronic lymphedema, usually in extremities; otherwise often in head and face
Other sites: Small intestine, ovary, kidney, lung, spleen
Key morphological features
Markers
  • Positive for endothelial markers factor VIII–related antigen, CD31, and CD34
Treatment
  • Early stage: wide excision
  • Later stage soft tissue: Preoperative radiation with or without chemotherapy followed by surgery
  • Bone: Neoadjuvant chemotherapy
  • Cutaneous: Surgery followed by radiation
Poor prognosis indicators
  • Older patient age
  • Retroperitoneum location
  • Larger tumor size
  • Bone or cutaneous angiosarcoma

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