Angiosarcoma

Author: Vanessa Ngan, staff writer, 2005.

What is an angiosarcoma?

Angiosarcoma is an uncommon malignant tumour (cancer) that arises from the cells that are normally used to make up the walls of the blood or lymphatic vessels. Haemangiosarcomas start in blood vessel walls and lymphangiosarcomas start in lymph vessel walls.

Angiosarcomas may occur in any organ of the body but are more frequently found in skin and soft tissue. They can also originate in the liver, breast, spleen, bone or heart.

What causes angiosarcoma?

The cause of angiosarcoma is usually unknown. The tumour may develop as a complication of a pre-existing condition. Certain patient groups may be at greater risk of developing angiosarcomas, these include:

What are the signs and symptoms of angiosarcoma?

The signs and symptoms of angiosarcoma differ according to the location of the tumour. Often symptoms of the disease are not apparent until the tumour is well advanced.

Affected organFeatures
Soft tissue
  • Rapidly growing tumour in the extremities or abdomen
  • Abdominal tumours may grow to large sizes before being detected as the abdomen can accommodate tumours
  • Haemorrhage, anaemia, haematoma, gastrointestinal bleeding
  • Adjacent lymph nodes enlarged
Skin
  • Enlarging bruise, a blue-black nodule, or unhealed ulceration
  • Lesions may bleed and be painful
  • Angiosarcomas occurring on the head and neck in elderly people are one of the most common forms of cutaneous angiosarcoma
Bone
  • Tumours may grow on multiple bones of the same extremity
  • Pain and tenderness of the affected area is common
  • Swelling and increased size of the affected limb may be present
Breast
  • Rapidly enlarging palpable mass without tenderness
  • Often there is no pain
  • Tumours often grow deep within breast tissue and cause diffuse breast enlargement with associated bluish skin discolouration
Other organs
  • Hepatic – non-specific symptoms such as fatigue, weight loss, right upper quadrant pain
  • Lung – chest pain, bloody sputum, weight loss, cough, difficulty breathing

Cutaneous angiosarcoma

Cutaneous angiosarcoma is the most common form of angiosarcoma not associated with chronic lymphoedema. The disease is primarily located on the head and neck of elderly persons and is also known as Wilson-Jones angiosarcoma, senile angiosarcoma or malignant angioendothelioma.

Clinical features of this form of angiosarcoma are:

What is the treatment of angiosarcoma?

Treatment of angiosarcoma is dependent on the location of the angiosarcoma and the extent of the tumour. Treatment includes chemotherapy, surgery, radiotherapy, or a combination of these treatment modalities.

The optimum treatment of cutaneous angiosarcoma has not been defined. These angiosarcomas are difficult to treat because of their multifocal nature and extensive spread pattern. Radical surgery and postoperative radiotherapy is generally used but tumour recurrence is common after treatment. Despite aggressive treatment, prognosis is poor in patients with cutaneous angiosarcomas.

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