What Is an Angiosarcoma?
An angiosarcoma is a cancerous tumor found in the inner walls of blood vessels and lymph vessels, which support the immune system. These vascular tumors can develop anywhere in the body, including organs. They most often occur in skin of the head and neck, in the liver or spleen, or in the chest or breast. They can also form in the heart, major blood vessels such as the aorta, and bones.
Angiosarcoma is very rare—each year, only one person in a million is diagnosed with the disease in the U.S. While anyone can develop one of these tumors, they are most common in people 70 and older.
Angiosarcomas are aggressive tumors that tend to grow quickly, metastasize (spread), and return in the area of surgery after removal. Unlike most other types of sarcoma, they can spread to a wide variety of locations in the body, including the brain. The most common locations of metastasis are the lungs, lymph nodes, bones, and other soft tissues.
The Sarcoma Program at Penn Medicine’s Abramson Cancer Center is home to one of the nation’s most experienced angiosarcoma care teams. We offer the latest treatment options for angiosarcoma through a team of coordinated specialists.
Angiosarcoma Symptoms
Small angiosarcoma tumors or those deep in the body might not cause any symptoms. However, tumors that grow larger or develop closer to the surface may cause:
- Noticeable lump
- Pain near the tumor
- Bruised area on the skin that looks purple, may grow larger, and can bleed when scratched or bumped
- Swelling near the bruise
Types of Angiosarcoma Cancer
Most types of angiosarcoma are classified as soft tissue sarcomas. In very rare cases angiosarcoma may also occur as a bone sarcoma. Angiosarcoma can take three forms:
- Conventional angiosarcoma: This form of angiosarcoma is by far the most common. Still, these tumors represent just 2 percent of soft tissue sarcomas.
- Epithelioid angiosarcoma: These tumors also typically form in soft tissues, but the cells take on a unique appearance. The tumors are also particularly aggressive.
- Angiosarcoma of bone: These rare tumors represent less than 1 percent of cancers starting in the bones. They occur most frequently in the arms or legs and most often affect adults ages 20 to 70.
What Causes Angiosarcoma?
Doctors often don’t know what causes an angiosarcoma to form. However, some factors can increase your risk of developing one:
- Chemical exposure: Previous exposure to chemicals such as vinyl chloride, thorium dioxide, arsenic, and radium may increase your chances of an angiosarcoma forming.
- Lymphedema: Certain problems with the lymphatic system—part of the larger immune system—can lead to swelling in arms or legs. In some cases, longstanding lymphedema may lead to an angiosarcoma forming.
- Family history: Some people inherit changes (also called variants or mutations) to a gene called POT1 from a parent. These changes can raise the risk of developing cardiac angiosarcoma.
- Radiation therapy: Previous radiation therapy for diseases such as breast cancer or Hodgkin lymphoma may increase the risk for angiosarcoma.
Angiosarcoma Diagnosis
Diagnosing angiosarcoma can be challenging because it can be difficult to tell where healthy tissue ends and cancerous tissue begins. Because these cancers are rare, not all doctors have experience with the nuances that are unique to angiosarcoma.
At Penn, evaluation of a possible angiosarcoma starts with a physical exam and a discussion of your symptoms. Imaging such as magnetic resonance imaging (MRI) provides more information, including tumor size and location. Computed tomography (CT) scans can also determine if the sarcoma has spread.
To confirm a diagnosis and gain more information, one of our specialized radiologists will take a sample of the tumor during an image-guided needle biopsy. After this biopsy, a specialized pathologist will examine the tissue sample under a microscope and conduct lab tests.
Together, the exam, imaging, and biopsy results will help your Penn Medicine team stage the angiosarcoma. This additional categorization helps guide our treatment recommendations. Learn more about sarcoma diagnosis and staging.
Angiosarcoma Treatment
Effective angiosarcoma treatment requires a team of specialists who work together. The main treatment for these tumors is surgery. Like sarcoma surgery for other tumor types, the best results come when the tumor is removed in one piece with a substantial buffer of normal tissue around the tumor. At Penn Medicine, our surgeons have extensive experience with this approach. The prognosis depends on:
- Tumor location
- Patient age and overall health
- Whether cancer has spread, and where it has spread to
- Ability of surgeons to completely remove cancerous tissue in its specific location
The location of angiosarcomas often makes them challenging to remove completely. If surgeons can’t remove enough normal tissue around the tumor, you may need radiation therapy to destroy any remaining cancer cells and help keep the cancer from returning. Your medical team may also recommend chemotherapy.
Even with effective treatment, angiosarcoma can often return in the area of surgery or spread elsewhere. Watching for that possibility requires ongoing checkups and regular imaging.
If angiosarcoma does spread or return, our doctors may recommend chemotherapy or targeted therapy—therapy aimed at particular aspects of cellular growth. Researchers are also exploring other options, including immunotherapy, which harnesses the immune system. Our clinical trials for Sarcoma are helping push care forward.
Learn more about our sarcoma treatment.
Make an Appointment
Please call 800-789-7366 or make an appointment.
The Penn Sarcoma Program offers same-day appointments to patients who have their imaging available. To learn more, call 215-349-8200.