Scientists still have a lot to learn about how this kind of cancer develops and why, but angiosarcomas can sometimes have a hereditary component. In the case of cardiac angiosarcomas specifically, scientists have located a gene, called POT1, that may make it more likely for a person to develop the disease, the NCI explains. Other potential risk factors for all kinds of angiosarcomas include a history of exposure to certain cancer-causing chemicals or radiation therapy, as well as lymphedema, a condition where lymph fluid builds up and causes swelling.
Symptoms of cardiac angiosarcoma depend on the location of the tumor. A cardiac angiosarcoma growing in the right atrium (the most common location) obstructs the flow of circulating blood in or out of the heart, Johns Hopkins Medicine explains. This can cause symptoms like swelling in the feet, ankles, legs, and abdomen, as well as bulging of the neck veins. An angiosarcoma that forms on the sac of tissue around the heart can cause fluid buildup within the sac, which can impede the heart’s ability to pump blood and trigger symptoms like chest pain, shortness of breath, and heart palpitations.
Other symptoms of cardiac sarcomas include coughing up blood and heart rhythm abnormalities. People can also experience more generalized symptoms, according to Johns Hopkins Medicine, like fever, weight loss, night sweats, and fatigue. And if small pieces of the tumor (called emboli) break off, they can be carried in the bloodstream to other regions of the body, obstructing blood flow and causing additional symptoms in that area. For example, emboli that travel to the lungs can cause respiratory issues.
Cardiac angiosarcomas can be diagnosed with a physical exam and a variety of diagnostic tests, including echocardiograms, electrocardiograms, imaging tests like CT scans, MRIs, and X-rays, blood tests, and biopsies, according to Johns Hopkins Medicine. Often, by the time a cardiac sarcoma begins causing symptoms and is diagnosed, it has already begun to spread to other parts of the body, which makes treatment more challenging.
The best treatment for a cardiac angiosarcoma depends on a variety of factors, like the size and location of the tumor and the medical history of the patient. Treatment options include open-heart surgery to remove the tumor, a heart transplant, or autotransplantation, a new procedure where the patient’s heart is temporarily removed (and the patient is put on a life-support machine) so that the surgeon can better excise the tumor. Chemotherapy and radiation therapy may also be part of treatment if cancer has spread throughout the body.
Currently, researchers are conducting clinical trials to develop new drug treatment therapies, according to Johns Hopkins Medicine. The more scientists study this disease, the better-equipped doctors will be to not only treat cardiac angiosarcoma, but also to diagnose and prevent it.