Angiosarcoma is a rare and clinically highly variable cancer of blood vessels (a form of sarcoma). High grade (aggressive) angiosarcomas can start anywhere in the body. The most common place for angiosarcomas to arise is in the head and neck area, breast (frequently several years after radiation and surgery for breast cancer), bone, or other vital organs such as liver and spleen. Less aggressive forms of angiosarcoma exist as well, such as epithelioid hemangioendothelioma (EHE). There are other rare forms of angiosarcoma, which will not be discussed here. Kaposi sarcoma is also a form of blood vessel sarcoma, but is discussed separately. Kaposi sarcoma is one of the rare forms of cancer caused by a virus, KSHV or HHV-8, which escapes control of the immune system as seen in patients with HIV or in elderly patients, typically living in the areas around the Mediterranean Sea.
Angiosarcomas are relatively rare. They comprise 1-2% of sarcomas in the surgical database from 1982-present from Memorial Hospital. Like other sarcomas, the risk of recurrence depends on the stage of disease. For sarcomas that are localized, low grade sarcomas are stage I, and large, high-grade, deep sarcomas are stage III. If a sarcoma does not have all three features (large, high-grade, and deep), it is stage II. Sarcomas that have traveled to lymph nodes or other sites of the body beyond where they started are considered stage IV, or metastatic, disease. Unlike most sarcomas, which travel via the bloodstream to other organs such as lung or liver, angiosarcomas are one form of sarcoma that travel to lymph nodes more often than most other sarcomas.
Distinct Clinical Features
Angiosarcomas tend to have two patterns of growth. In one form they form hard white nodules; in the other, they form blood-filled blebs (looking something like blood blisters) that can be multiple in an area, and grow and spread in an irregular pattern near the surface of the skin, or as masses in soft tissue or other organs. Angiosarcomas have the same proteins within them that are found in blood vessels; they usually contain blood vessel marker proteins CD31 and CD34. As a result they are usually easy to distinguish. It is necessary to distinguish angiosarcomas from hemangiomas, which are non-malignant (benign) collections of blood vessels.
Treatment and Follow-up for Localized Disease
Angiosarcomas have a particular ability to recur near the site the tumor first started. As a result, it is more difficult to achieve a clean margin around these tumors at the time of a surgery. For the primary treatment of these tumors, surgery is most often employed, and radiation is often added to try to control the tumor locally. There is no evidence giving chemotherapy after surgical removal of the tumor increases one’s chance for survival. Treatment is also often made more difficult in the breast when the tumor follows a course of radiation; it is often possible to surgically remove the remaining tumor, but it becomes too dangerous to give radiation to the same area twice, the risk being the permanent damage of normal tissue after such radiation. In these situations surgical resection alone is usually recommended.
Treatment and Follow-up for Metastatic Disease
In the situation where the tumor is recurrent or metastatic, in some cases radiation or another surgical resection can be offered, but usually treatment consists of intravenous chemotherapy directed against the tumor. The best commercially available chemotherapy drugs for angiosarcomas in our experience at MSKCC have been treatments that contain doxorubicin (including Doxil/Caelyx) or taxanes (docetaxel or paclitaxel). It is possible with careful dose adjustment to be able to treat people with metastatic disease for a year or more with a single type of treatment.