Angiosarcoma (syn. malignant haemangioendothelioma)

Cutaneous angiosarcomas are rare, aggressive malignant endothelial-cell tumours of vascular or lymphatic origin.

This chapter is set out as follows:

• Angiosarcoma makes up 4.16% of all soft tissue sarcomas
   
• Although angiosarcoma can affect any age, the median age at diagnosis is 71 years old. There are slightly more females than males affected 
   
• In most cases the cause is unknown, although a history of excessive sun exposure is common
   
• Angiosarcoma can can occur secondary to:
  • Chronic lymphoedema (Stewart-Treves syndrome) 
  • Previous radiotherapy - angiosarcomas of the breast are more likely to be found in women who have had radiotherapy to treat breast cancer

Distribution

• The head and neck is the most common site for primary lesions, although they can occur anywhere on the skin or within the body

Morphology

• The initial presentation may be subtle with bruises / thin purple patches and plaques
• Lesions become more nodular and ulcerate with time
• Metastases are common and lesions on limbs are particularly aggressive

• Management is in Secondary / Tertiary care - suspected cases should be referred on an urgent skin cancer (or sarcoma) pathway  
   
• Surgical treatment is first-line. Other treatment options include radiotherapy, chemotherapy, and immunotherapy
   
• Prognosis:
  • 62% of people diagnosed with angiosarcoma in England survive their disease for one year or more (2013-2017)
  • 32% of people diagnosed with angiosarcoma in England survive their disease for five years or more (2013-2017)