Cardiac Angiosarcoma at a Glance

Overview

Cardiac angiosarcoma (heart cancer) is a rare malignant tumor that originates in the endothelial cells that

line the blood vessels of the heart (Kumari et al., 2023). It predominantly affects both the atrium and

ventricle of the right side of the heart as well as the pericardium (Cleveland Clinic, 2024). Although it

only accounts for around 1% to 2% of all sarcomas, its impact is devastating. Cardiac angiosarcoma has a

poor prognosis, with survival rates of 10-16 months (Harris, 2021; Nall, 2020). Limited research and late

diagnosis are attributed to this prognosis. However, there may be some actions individuals can take that

may lower their risk for cardiac angiosarcoma.

Cause/Research

Due to the small sample size, research on this topic is limited, and thus, the cause of cardiac

angiosarcoma is unclear. A 2015 study postulates that a mutation in the POT1 gene is a plausible cause

for this cancer. POT1 binds to telomeres as a part of the shelterin complex, which regulates telomerase

activity and chromosome stability (Calvete et al., 2015). A mutation of this protein would result in

elongated telomeres and telomere instability (Calvete et al., 2015). A telomere is a repeated sequence of

DNA that protects genetic data and regulates the life cycle of a cell. So, a mutation to the POT1 protein

could cause the immortalization of cells that are now more susceptible to mutations in critical genes.

Diagnosis/Treatment

Cardiac angiosarcoma’s clinical presentation is diverse and nonspecific, which leads to late diagnosis

(Kumari et al., 2023). Compounded with limited research and the typical rapid development of the

sarcoma, treatment of this condition is difficult. There is no standard of treatment for cardiac

angiosarcoma (Harris, 2021). Instead, treatments from other sarcomas are applied (Harris, 2021).

Treatment can include surgery, chemotherapy, and/or radiation. Optimal chemotherapy regimens have not

been discerned yet, which is one of many factors illustrating the need for more research on this topic

(Kumari et al., 2023).

Preventative Measures

Researchers have yet to determine preventive methods for cardiac angiosarcoma. However, there may be

some actions an individual may take to lower their risk. One would be to limit their exposure to known

carcinogenic chemicals (Cleveland Clinic, 2024). Limiting your exposure to secondhand smoke and

refraining from smoking may help (Nall, 2020). Screening for genetic irregularities may

also help catch the disease early (Cleveland Clinic, 2024).

Takeaways

Limited research and the nonspecific clinical presentation of cardiac angiosarcoma are primarily

responsible for the difficulty in diagnosis and treatment. Furthermore, this difficulty, along with the rapid

development of the cancer, leads to poor prognosis. General cancer preventative measures can be taken,

but there is no research on specific measures for cardiac angiosarcoma. It is critical to

engage in more research on this topic.