Social media flooded with tributes to fashion designer Virgil Abloh after it was announced in November 2021 that the 41-year-old had died from a rare form of cancer.

“We are devastated to announce the passing of our beloved Virgil Abloh, a fiercely devoted father, husband, son, brother, and friend,” read the caption of a post on Abloh’s Instagram account. “He is survived by his loving wife Shannon Abloh, his children Lowe Abloh and Grey Abloh, his sister Edwina Abloh, his parents Nee and Eunice Abloh, and numerous dear friends and colleagues.”

The post explained that Abloh “valiantly battled a rare, aggressive form of cancer” called cardiac angiosarcoma and that he did so behind closed doors. “He chose to endure his battle privately since his diagnosis in 2019, undergoing numerous challenging treatments, all while helming several significant institutions that span fashion, art, and culture,” the post said.

“Through it all, his work ethic, infinite curiosity, and optimism never wavered. Virgil was driven by his dedication to his craft and to his mission to open doors for others and create pathways for greater equality in art and design,” the post read. “He often said, ‘Everything I do is for the 17-year-old version of myself,’ believing deeply in the power of art to inspire future generations.”

Abloh was the artistic director of Louis Vuitton’s menswear collection from 2018 until his death and also served as the CEO of the Milan-based label Off-White, his own fashion house. His death has brought up a lot of questions about cardiac angiosarcoma. Here’s what you need to know.

Angiosarcoma is a rare cancer that develops in the inner lining of blood vessels and lymph vessels. Angiosarcomas can develop anywhere in the body, but they usually occur in the skin, breast, liver, and spleen.

Cardiac angiosarcoma happens in the chambers of the heart, said Jack Jacoub, MD, medical oncologist and medical director of MemorialCare Cancer Institute at Orange Coast Medical Center in Fountain Valley, California.

A Rare Disease

Angiosarcomas as a whole are diagnosed in about one in 1 million people in the US each year, making them incredibly rare. And each type of angiosarcoma, including cardiac angiosarcoma, is even rarer.

“A very busy cancer doctor might see one or two [cases] in their whole career,” Dr. Jacoub said.

Authors of a 2019 case report published in Surgical and Experimental Pathology pointed out that cardiac angiosarcoma can be difficult to diagnose, given that the initial symptoms are usually “non-specific.” Dr. Jacoub agreed and said there are a few things that someone might notice with this form of cancer:

• Fainting
• Arrhythmia (where the heart beats too fast or too slow)
• Dizziness
• Blood pooling in the legs and ankles, leading to swelling
• Chest pain
• Coughing up blood

The symptoms of cardiac angiosarcoma are similar to symptoms of many other health conditions, according to a 2022 case report. Only a healthcare provider can tell if you have cancer, so it’s important to get medical attention if you have any of these symptoms.

Given how rare cardiac angiosarcomas are, it’s difficult for scientists to parse out exactly what the risk factors for this particular form of cancer are, Dr. Jacoub said. “There’s not a certain lifestyle scenario that specifically raises your risk of cardiac angiosarcoma, like smoking and lung cancer,” explained Dr. Jacoub.

Ricardo Gonzalez, MD, chair of the Sarcoma Department at Moffitt Cancer Center in Tampa, Florida, said that “most of these are spontaneous while a very small number may be associated with genetic syndromes.”

A change in a gene called POT1 may predispose people to cardiac angiosarcoma, and it can be inherited through families.

Other risk factors are those that increase cancer risk in general, Dr. Jacoub said, including having past radiation therapy or exposure to some cancer-causing chemicals.

The condition is usually diagnosed when someone visits their doctor after having unusual symptoms, like fainting or dizziness, Dr. Jacoub said. To test for angiosarcoma, a healthcare provider can use imaging tests such as MRI, CT, or PET scan and may perform a biopsy, if needed.

“At times, the tumor can look like a clot, which is vastly more common,” Dr. Jacoub said, making this a difficult condition to diagnose. Once a patient is properly diagnosed, healthcare providers will usually recommend surgery to try to remove the tumor. “That’s really the best chance someone has,” Dr. Jacoub added.

That will often be followed by chemotherapy to try to kill off any cancer cells that may remain.

Given how difficult it can be to operate on the chambers of the heart and because the cancer may have spread to other areas within the heart, Dr. Jacoub said that healthcare providers may recommend a heart transplant in some cases. But, he pointed out, this isn’t common. “Unfortunately, there really isn’t a standard of care with cardiac angiosarcoma,” Dr. Jacoub explained.

The management steps will really depend on how far the cancer has spread, said Teresa Lee, MD, PhD, an assistant professor at Fox Chase Cancer Center in Philadelphia. “Because nonspecific symptoms can be common at first, cardiac angiosarcoma is often spread to other places in the body by the time it is discovered. In this case, treatments, including possibly chemotherapy or radiation, are aimed at relieving symptoms and hopefully prolonging survival by a few months,” added Dr. Lee.

If caught early, surgery—either removal of the tumor or an actual heart transplantation—may be curative, according to Dr. Lee.

But usually, cardiac angiosarcoma is a deadly disease. “Even with surgery, the prognosis is usually disappointing,” Dr. Jacoub said. “The average survival after diagnosis is usually somewhere between six to 12 months.”

“In the best of circumstances, patients may live a few years after surgery, chemotherapy, and radiation,” Dr. Gonzalez said. “Most patients will live under one year. There are no absolutes in medicine; however, I would consider this a largely a terminal diagnosis unless it is identified very early and the patient doesn’t develop metastatic disease over the period of follow-up.”

Angiosarcoma is a rare, but deadly disease. It can be difficult to diagnose since symptoms are often non-specific. But some symptoms have been associated with this kind of cancer. If you experience symptoms such as fainting, arrhythmia, dizziness, or chest pain, talk to your healthcare provider.