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.
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.
Signs and symptoms of angiosarcoma
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:
- A noticeable lump
- Pain near the tumor area
- A purple, bruise-like mark on the skin that may grow or bleed easily
- Swelling near the discolored skin
Risk factors and causes of 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.
Getting an accurate angiosarcoma diagnosis
Diagnosing angiosarcoma can be challenging because it’s difficult to tell where healthy tissue ends and cancer begins. Because these tumors are rare, not all doctors have experience with their unique traits. At Penn Medicine, your evaluation starts with a physical exam and a review of your symptoms.
Imaging scans provide information about tumor size, location, and whether the sarcoma has spread. To confirm the diagnosis, a radiologist may take a small tissue sample using an image-guided needle biopsy. After this biopsy, a pathologist then studies the tissue cells under a microscope. These combined results help your care team stage the angiosarcoma and guide treatment recommendations.
Your options for angiosarcoma treatment
Surgery is the main treatment for these tumors. The best outcomes occur when a surgeon removes the tumor in one piece, along with a margin of healthy tissue around it. How well this works often depends on your age, your overall health, the tumor’s location, and whether the cancer has spread.
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. Your team may also suggest powerful cancer-fighting medicines to help keep the disease from returning.
Even with effective treatment, angiosarcoma can often return and spread. Watching for that possibility requires ongoing checkups and regular imaging. If the cancer does return, your team can use targeted therapies that block tumor growth. Researchers are also exploring other options like immunotherapies that prompt your own immune system to fight back. At Penn Medicine, clinical trials for sarcoma are helping push care forward.
Why experience matters for rare cancers
Angiosarcoma is rare enough that many cancer centers see only a handful of cases each year. At Penn Medicine, our sarcoma program is one of the most comprehensive in the region, which means our team has extensive, hands-on experience with these complex tumors.
Our dedicated sarcoma specialists include surgeons, oncologists, radiologists, and pathologists who work closely together on your care. Each week, this team meets during tumor board conferences to review cases, share expertise, and build personalized treatment plans. When appropriate, they also consider the latest clinical trials and emerging therapies, giving you access to options that may not be available elsewhere.
Rated “exceptional” by The National Cancer Institute
Penn Medicine’s Abramson Cancer Center is a world leader in cancer research, patient care, and education. Our status as a national leader in cancer care is reflected in our continuous designation as a Comprehensive Cancer Center by the National Cancer Institute (NCI) since 1973, one of 7 such centers in the United States. The ACC is also a member of the National Comprehensive Cancer Network, one of a select few cancer centers in the U.S., that are working to promote equitable access to high-quality, advanced cancer care.