What is Cardiac Angiosarcoma?

On Nov 28, Virgil Abloh, a noted designer and artistic director of men’s fashion at Louis Vuitton, died at 41 in Chicago after a two-year battle with cardiac angiosarcoma. According to a statement released on his Instagram account, Abloh was diagnosed with the rare cancer in 2019 and underwent a number of treatments before he died.

WHAT IS CARDIAC ANGIOSARCOMA?

First described by doctors in 1934, cardiac angiosarcoma is a rare form of cancer that causes cells inside the blood vessels of the heart to multiply and form tumors. The cancerous cells are often found in blood vessels lining the musculature of the wall of the heart, said Dr Robert Maki, a medical oncologist and the clinical director of the Sarcoma Program at Penn Medicine in Philadelphia.

These cancers are part of a larger family of cancers called sarcomas, which affect the connective tissues that support and surround other parts of the body, including muscle, fat and blood vessels. Angiosarcomas are a kind of sarcoma that specifically afflict cells that line blood vessels, and cardiac angiosarcomas are angiosarcomas that occur within or around the heart.

Cardiac angiosarcomas that begin in the heart are known as primary cardiac angiosarcomas, whereas cancers that form elsewhere and then spread to the heart are secondary cardiac angiosarcomas. It’s unclear which kind Abloh had, but it’s likely it was primary, said Dr Sumeet Mitter, a cardiologist at The Mount Sinai Hospital in Manhattan, because if the tumour had originated elsewhere it would probably be described differently.

Angiosarcomas are aggressive and can also spread, or metastasise, to other parts of the body, including the lungs, liver and bone marrow, Dr Maki said.

HOW COMMON ARE CARDIAC ANGIOSARCOMAS?

Cardiac angiosarcomas are extremely rare. Of the 17,000 or fewer people in the United States who are diagnosed with sarcomas each year, up to 700 are diagnosed with angiosarcomas, and then only 30 to 40 of those have cardiac angiosarcomas, Dr Maki said.

Angiosarcomas are more common in dogs, especially retrievers, Dr Maki added, where they often afflict the spleen.

WHAT ARE THE SYMPTOMS?

Cardiac angiosarcomas can be difficult to identify because people often do not have symptoms, or they have only vague symptoms like shortness of breath or fatigue during physical exertion, said Dr Ankit Mangla, a medical oncologist at the Case Comprehensive Cancer Center in Cleveland. These symptoms typically arise because the tumours make it harder for blood to flow through the chambers of the heart.

But while many people experience shortness of breath and fatigue, only a very small portion of people with these symptoms have cardiac angiosarcoma. Typically, people have other heart problems instead — the heart muscle itself might be weak, or it may be too muscular and stiff, Dr Maki said.

HOW ARE CARDIAC ANGIOSARCOMAS DIAGNOSED?

Diagnosing these rare cancers can be difficult, because the tumours cannot always be seen using common imaging techniques such as CT scans, which are often administered to patients in emergency rooms who complain of shortness of breath, Dr Mangla said.

During a physical exam, a doctor might, however, be able to hear the sounds of blood rushing around the tumour. They might then conduct an ultrasound of the heart to visualise and identify a tumour, and then follow up with other kinds of imaging, including MRI, PET scan or three-dimensional echocardiography.

HOW DO DOCTORS TREAT CARDIAC ANGIOSARCOMA?

It depends on the patient, but treatment might include surgery to try to remove the heart tumour, or a heart transplant. But sometimes — even in transplant patients — the tumour can reappear.

Generally, if the cancer has not yet spread, it won’t be treated with chemotherapy or radiation. “It’s very rare for us to be able to treat just the heart itself with something like chemotherapy, and you also don’t want to necessarily radiate the heart either, because that will damage it,” Dr Maki said.

If the cancer has spread, though, a patient might undergo chemotherapy, radiation or treatment with other cancer-fighting drugs that can kill the spreading cancer.

It is unclear whether Abloh’s cancer had spread, but if he had it for two years, that is certainly possible, Dr Maki said.

WHAT IS THE PROGNOSIS?

Because cardiac angiosarcomas can damage the heart, which is a vital organ, and because they are aggressive and often spread, these cancers are often fatal.

Some research suggests that if people do not undergo surgery, or their surgery does not remove all of the cancer, they typically survive for up to nine months, Dr Mangla said. With surgery and other treatments, patients may live for 12 to 30 months.

WHAT ARE THE RISK FACTORS FOR CARDIAC ANGIOSARCOMA?

Because so few people are diagnosed with this kind of cancer, we don’t know much about specific risk factors, Dr Maki said.

However, people are more likely to be diagnosed with angiosarcomas in general if they have undergone radiation therapy or have been exposed to high levels of vinyl chloride (a chemical used to make PVC); arsenic; or thorium dioxide, a chemical people can be exposed to in drinking water if they live near sites that mine for the radioactive metal thorium or use it in manufacturing.

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