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 Putting the Extremely Rare Cardiac Angiosarcoma in the Spotlight

Cardiac angiosarcoma is an exceptionally rare cancer that has become national news because of the recent death of acclaimed fashion designer, Virgil Abloh. He was 41 years old when he died in November 2021, following a 2-year battle with the disease, which is an aggressive subtype of soft-tissue sarcoma. Angiosarcomas develop in vascular endothelial cells and can grow along preexisting vascular channels. They can also form poorly organized vessels, solid masses, or nodules. An estimated 90% of cardiac angiosarcomas appear as a multicentric mass in the right atrium of the heart.

Articles of Interest

Cardiac angiosarcoma: A diagnostic and therapeutic challenge

Cardiac angiosarcomas are a rare group of soft tissue sarcomas, characterized by aggressive local growth and early spread. Because this is an uncommon disease, there is currently no standard treatment approach. When localized, surgery appears to lead to the best outcomes, but this can be technically challenging and not always feasible. Upfront chemoradiotherapy provides an alternative that may shrink the tumor to enable definitive surgical resection.

Cardiac Sarcomas: An Update

Primary cardiac sarcomas are rare and represent 20% of all primary cardiac tumors. Symptoms depend on the chambers and the cardiac structures involved. Transthoracic echocardiography is commonly used to identify a cardiac mass. The diagnosis of cardiac sarcoma requires adequate sampling and the careful use of ancillary diagnostic techniques.

Heart cancer: Insights on diagnosis, treatment and research

Although sarcomas of the heart are rare, most cases are a subtype called cardiac angiosarcoma. They tend to develop from the cells that line the right atrium and rarely occur in the left chambers of the heart.

Heart Cancer: Is There Such A Thing?

Yes. Most tumors that form in the heart aren't cancerous. But some heart tumors can be cancerous. Cancer that begins in the heart is most often sarcoma, a type of cancer that originates in the soft tissues of the body.

Primary Cardiac Angiosarcoma: A Review

Primary cardiac angiosarcoma is a rare and aggressive malignancy originating from the endothelial lining of cardiac blood vessels. This review covers various aspects of the disease, including its pathogenesis, clinical presentation, diagnosis, treatment, and prognosis. The primary characteristic of cardiac angiosarcoma is the rapid growth of abnormal blood vessels that invade the heart muscle, leading to the destruction of healthy tissue. Due to its infiltrative nature and early spread, diagnosing and treating cardiac angiosarcoma present significant challenges.

The heart isn't particularly vulnerable to cancer – and here's why

Most heart tumors – about 75 percent – are benign, meaning they are not cancerous. Cancer that spreads to the heart from the lung, breast or elsewhere in the body is much more common. But when tumors begin in the heart, they "are likely a result of genetic malformations, since the heart is not a common place for cancers to grow," Leja said.

What is angiosarcoma?

Angiosarcoma is a rare cancer that originates in the endothelial cells lining blood or lymph vessels. These cells typically form the barrier between the bloodstream and surrounding tissues. In angiosarcoma, these cells become malignant, multiplying uncontrollably and forming new, abnormal blood vessels that eventually lead to tumor development. This cancer is known for its aggressive nature, often leading to a high rate of metastasis, where cancer cells spread to other parts of the body.

What Is Cardiac Angiosarcoma?

Virgil Abloh, the celebrated fashion designer, died at 41 after being diagnosed with the rare cancer. Here’s what we know about it.

Resources

StatPearls

Primary cardiac tumors are much rarer than secondary malignant lesions, occurring in about 0.001 to 0.3% of autopsies. Almost 90% of primary cardiac tumors excised surgically are benign.