Angiosarcoma

Author: Vanessa Ngan, Staff Writer, 2005. Updated by Dr Ebtisam Elghblawi, Tripoli, Libya, April 2017.

What is an angiosarcoma?

Angiosarcoma is an uncommon and aggressive malignant tumour (cancer) that arises from endothelial cells, which normally line the walls of blood or lymphatic vessels.

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.

After they have been surgically excised, angiosarcomas:

Who gets angiosarcoma?

Angiosarcoma is twice as common in males than in females. Angiosarcoma of the head and neck mostly affects older people.

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 causes angiosarcoma?

The cause of the rapid proliferation and infiltration of malignant endothelial cells in angiosarcoma is usually unknown. 

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.

Soft tissue

Skin

Bone

Breast

Other organs

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.

Paclitaxel is potent antiangiogenic drug that shows promise in the management of angiosarcoma when given weekly.

Related information

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