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
- Reddish-brown masses
- Commonly multicentric
- Sites commonly with specific risk factors
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
- Infiltrative growth
- Anastomosing vessels lined by atypical endothelial cells
- Blood may be seen in spaces, except for lymphangiosarcoma
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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