Angiosarcoma is a rare type of soft tissue sarcoma accounting for about 1% of soft tissue sarcoma diagnoses. It usually occurs in the head and neck area but can arise from any blood or lymphatic vessel. Prior radiotherapy treatment is a significant risk factor for development of this tumour (5-20% of cases).
Epithelioid Haemangioendothelioma is a less aggressive sarcoma with a strong propensity for local recurrence. It is often treated as angiosarcoma locally.
Diagnosis
Patients present with a blue or purple mass that grows over weeks to months. Most cases arise in the skin or subcutaneous tissue but may rarely arise in a deeper structure, often associated with a vessel. There may diffuse involvement of the skin and subcutaneous tissues as well as deeper invasion. Unlike most soft tissue sarcomas, angiosarcoma may spread to regional lymph nodes (10% at diagnosis).
Epithelioid haemangioendothelioma may present with a painful nodule associated with a vessel; patients may develop distal thrombus which may also be symptomatic.
Treatment
Wide local surgical excision is the primary form of treatment. Given the propensity for occurence in the head and neck area this can be disfiguring.
Adjuvant radiotherapy has been shown to improve local control and overall survival in small series (overall survival 70% vs 30%). The role for neoadjuvant therapy or cytotoxic therpay at any stage is not established.
Outcomes
5 year survival is about 30% and 10 year survival 15%. Both local recurrence and distant metastases are common with angiosarcoma; survival is signifincantly better with epithelioid haemangioendothelioma.
