Soft tissue sarcoma

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Blood and lymph vessel tumours

Cancerous tumours of the blood and lymph vessels may also be called malignant vascular tumours. Several different types of soft tissue sarcoma can develop in the blood or lymph vessels, including those below.

Kaposi sarcoma is an uncommon tumour that also develops in the blood vessels. Find out more about Kaposi sarcoma.

Angiosarcoma

Angiosarcoma is a rare type of soft tissue sarcoma that starts in the cells of the blood vessels. It accounts for about 1% of all soft tissue sarcomas. It can develop in the skin, liver, breast, bone, abdomen, retroperitoneum (the back of the abdomen) or heart.

Angiosarcomas usually develop in areas where there is a buildup of lymph fluid, which is called lymphedema. Lymphedema can happen after radiation therapy if this treatment damages lymph vessels. For example, some women who develop lymphedema after radiation therapy for breast cancer have a risk for developing angiosarcoma.

These tumours can also develop when lymphedema isn’t present. For example, women who receive radiation therapy for cancers of the reproductive organs have a risk of developing angiosarcoma in the pelvis.

Angiosarcomas are usually high grade, which means they grow quickly and are likely to spread, or metastasize, to other parts of the body. Because these tumours start in blood vessels, they can easily spread into the bloodstream and to lymph nodes and other organs. They tend to come back, or recur, in the same area as the original tumour. This is called local recurrence.

Hemangioendothelioma

Hemangioendothelioma is a soft tissue sarcoma that can start in blood vessels close to the surface of the body (superficial). It can also develop in soft tissues deep in the body, including internal organs such as the liver or lungs.

These tumours are low grade, which means they grow slowly. They are considered less aggressive, but they may grow into nearby tissues and can sometimes spread to other parts of the body.

Hemangiopericytoma

Hemangiopericytoma is also called a perivascular tumour. It starts in cells that surround blood vessels. This tumour is most common in adults and often starts in the legs, arms or pelvis.

Hemangiopericytomas can be either non-cancerous (benign) or cancerous (malignant). Most are benign and slow growing. But some are more aggressive, which means they can grow and spread quickly. Because hemangiopericytomas can be aggressive, they are often treated as cancerous. They are usually removed with surgery and you will have long-term follow-up to check if they have come back or spread.

Lymphangiosarcoma

Lymphangiosarcoma is sometimes considered a subtype of angiosarcoma. It develops in the lymph vessels.

This type of tumour tends to occur in areas of the body with constant buildup of lymph fluid, which is called chronic lymphedema or Stewart-Treves syndrome. Chronic lymphedema may be caused by surgery or radiation. It may also be related to certain diseases that are present at birth (congenital diseases) or inflammatory diseases.

Lymphangiosarcoma is often linked with radiation therapy and lymph node removal to treat breast cancer.

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