Cardiac Angiosarcoma: Symptoms, Causes, and Treatment of This Rare Heart Cancer
Updated: 2 days ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026
Quick answer
Cardiac angiosarcoma is a rare but aggressive heart cancer that starts in the lining of the heart's blood vessels and spreads into the heart muscle [1] [5], with about 80% of cases beginning in the right atrium [1]. Symptoms include shortness of breath and chest pain [1]. Surgery to remove the whole tumor offers the best chance of survival [1], but the outlook is poor [1] [5].
What the research says
According to PubMed:
Cardiac angiosarcoma is about a quarter of primary heart cancers, and surgery was linked to better survival. In U.S. SEER registry data (2000–2014), 497 patients had primary malignant cardiac tumors, and angiosarcomas made up 27.3%. Cardiac angiosarcoma incidence showed no significant change over the study period, its survival was significantly worse than for cardiac non-Hodgkin lymphoma (P<.001), and surgical treatment was associated with significantly better overall survival (P=.027) (Saad et al., Cardiovascular Pathology, 2018) [6].
Several imaging tests work together to diagnose it. A review reports that transesophageal echocardiography is highly sensitive for cardiac tumors, while CT and cardiac MRI help assess tumor anatomy and look for metastases. Because the disease is usually advanced at diagnosis, the prognosis is generally poor, and the authors stress a multidisciplinary approach (Kumari et al., Cureus, 2023) [5].
It can first show up as fluid around the heart. In a case report of a patient whose cardiac angiosarcoma presented as cardiac tamponade, the authors note that dyspnea is the most common but non-specific presenting symptom, and that spread often rules out surgery — which is why early diagnosis matters (Elmusa et al., Cureus, 2022) [3].
Study details are from PubMed abstracts.
What is cardiac angiosarcoma?
Cardiac angiosarcoma is a malignant (cancerous) tumor in your heart. It starts in the lining of your heart's blood vessels and spreads to your heart muscle [1] [5].
An invasive and aggressive tumor, it spreads to your heart muscle. This rare type of cancer often moves to other parts of your body before a diagnosis happens [1]. Because of this, it's difficult to treat cardiac angiosarcoma.
In 80% of cases, this rare tumor happens in your right atrium (upper heart chamber) [1]. It can also show up in your right ventricle (lower heart chamber) or pericardium (the protective sac around the outside of your heart). Rarely, it can be in the left side of your heart [1].
This aggressive, invasive tumor can block or push against areas (like blood vessels and valves) around it. The invasiveness of this tumor can make it hard to remove with surgery [1].
Where it can start | How often |
Right atrium (upper heart chamber) | About 80% of cases |
Right ventricle (lower heart chamber) | Can also occur |
Pericardium (protective sac around the heart) | Can also occur |
Left side of the heart | Rare |
How common is cardiac angiosarcoma?
Autopsy results have shown that fewer than 0.03% of people had a primary cardiac neoplasm (abnormal tissue growth) [1]. As part of that category, cardiac angiosarcoma is rare. But it's the most common kind of malignant tumor an adult can get in their heart [1] [7].
People usually get a diagnosis before age 65, usually between their 20s and 40s [1]. These tumors happen two to three times more often in males [1].
What are the symptoms of cardiac angiosarcoma?
Cardiac angiosarcoma symptoms vary depending on the location of the tumor. Symptoms may include shortness of breath (dyspnea), chest pain, fatigue, syncope (passing out), and heart palpitations [1].
If the tumor spreads beyond your heart, you may have other symptoms, like anemia or weight loss [1].
What causes cardiac angiosarcoma?
Cardiac angiosarcoma has no clear cause, although some factors may make people more likely to develop it. But people with certain mutations (changes) in their DNA, like the POT1 gene, may be more likely to get cardiac angiosarcoma [1] [2]. Another possibility is that people who get this disease received radiation therapy to their chest to treat childhood cancer [1].
What are the risk factors?
Risk factors for cardiac angiosarcoma include a history of radiation therapy for treatment of another cancer, exposure to certain chemicals that can cause cancer, and lymphedema [1] [2].
What are the complications of cardiac angiosarcoma?
Complications of cardiac angiosarcoma may include heart failure, blockage in your vena cava (large blood vessel), arrhythmia (abnormal heart rhythm), pericardial effusion, cardiac tamponade, heart valve regurgitation (leak), and obstructive shock [1].
How is cardiac angiosarcoma diagnosed?
Cardiac angiosarcoma can be diagnosed with heart imaging such as echocardiograms, CT, MRI and PET scans, along with cardiac catheterization and a heart biopsy [1].
Transthoracic echocardiogram (noninvasive)
Transesophageal echocardiogram (invasive)
Computed tomography (CT)
Heart MRI (magnetic resonance imaging)
Positron emission tomography (PET)
Cardiac catheterization
Heart biopsy
How is cardiac angiosarcoma treated?
Surgery can improve your symptoms [1]. Removing the whole tumor gives you the best chance of surviving [1]. But it can be difficult to remove the entire tumor if it's spread to other areas of your heart. Choosing a surgeon who has experience with this kind of rare tumor may increase your chances of a complete tumor removal [1].
Chemotherapy can ease your symptoms. It can also help some people live longer [1]. Sometimes, radiation can improve your situation [1].
What are the complications of treatment?
Complications of surgery may include bleeding, abnormal heart rhythm (arrhythmia), and heart dysfunction [1].
Can I lower my risk of cardiac angiosarcoma?
You may be able to lower your risk of cardiac angiosarcoma by limiting your exposure to certain chemicals [1]. If you have a genetic irregularity that puts you at risk for this kind of cancer, talk to your provider. You may be able to get screenings that could catch cardiac angiosarcoma early [1].
What is the outlook?
The outlook for cardiac angiosarcoma is very poor because the cancer aggressively invades nearby areas, often spreads before diagnosis and can return after treatment [1].
What can I expect if I have cardiac angiosarcoma?
A tumor can return after treatment. It's common for cardiac angiosarcoma to spread to your liver, lungs and brain before you get a diagnosis [1]. It can also spread to your bones and adrenal glands [1]. This is what makes it a difficult cancer to treat and survive.
What is the prognosis?
The cardiac angiosarcoma prognosis is very poor [1] [5]. The disease aggressively invades the areas around it. Cardiac angiosarcoma that spreads (metastasizes) to other body parts is often fatal [1].
How can people live with cardiac angiosarcoma?
Living with cardiac angiosarcoma involves eating nutritious foods, getting enough rest, finding ways to relax and support, and keeping all scheduled appointments with your provider.
How do I take care of myself?
The basic things you do when you're healthy are still important when you're fighting cancer. Eating nutritious foods ensures your body's getting the nutrition it needs. Getting enough rest is even more important if treatments make you tired.
If you need to relax, try yoga, meditation or deep breathing. You may also want to find a support group (even if it's online) where you can safely share what you're feeling.
When you're nearing the end of your life, hospice care can help keep you comfortable.
When should I see my healthcare provider?
Keep all scheduled appointments with your provider. But don't hesitate to contact them if your symptoms come back, your symptoms get worse, or you need help with side effects from treatment.
When should I go to the ER?
Emergency: Get emergency help if you have shortness of breath, chest pain, or a fever or other signs of infection if you've had chemotherapy [1].
What questions should I ask my doctor?
Since cardiac angiosarcoma is rare, it may be hard to find much information about it. You may want to ask your healthcare provider:
Has this cancer spread to other parts of my body?
Is surgery an option for me?
From looking at imaging, does it seem likely that you can remove the whole tumor?
Which treatments do you recommend for me?
What's the outlook for someone in my situation?
A note from the experts. Most people have never heard of cardiac angiosarcoma. It can be difficult to explain your situation to your family and friends. Don't be afraid to ask your provider about anything you don't understand. Take advantage of any resources or services available to help you. If you don't have a good prognosis, talk to someone you trust about what you want. It may give you comfort to talk to a counselor and arrange hospice services.
Conclusion
Cardiac angiosarcoma is a rare and aggressive malignant tumor that begins in the lining of the heart's blood vessels and invades the heart muscle — usually in the right atrium, and usually in adults between their 20s and 40s. Because it often spreads to the liver, lungs, brain, bones, or adrenal glands before it is ever diagnosed, it is one of the most difficult heart cancers to treat and survive. There is no clear cause, though DNA mutations (like the POT1 gene) and prior chest radiation are possible contributors. Complete surgical removal offers the best chance of survival, supported by chemotherapy and sometimes radiation. The prognosis is poor, which makes early attention to symptoms — shortness of breath, chest pain, fainting, fatigue, and palpitations — and honest conversations with your care team essential. If you or someone you love is facing this diagnosis, lean on your provider, ask the hard questions, take care of your body and your rest, and do not hesitate to seek support from counselors, support groups, or hospice services.
Looking for clear answers on tough health questions? Browse hundreds of plain-language guides at Rinnit.com.
Frequently asked questions
What is cardiac angiosarcoma?
Cardiac angiosarcoma is a malignant (cancerous) tumor in your heart. It starts in the lining of your heart's blood vessels and spreads to your heart muscle.
Is cardiac angiosarcoma common?
No. Fewer than 0.03% of people have any primary cardiac tumor. But it is the most common kind of malignant tumor an adult can get in their heart.
Where in the heart does it start?
In about 80% of cases it happens in the right atrium (upper heart chamber). It can also show up in the right ventricle or the pericardium (the protective sac around the heart), and rarely on the left side of the heart.
Who is most likely to get it?
People usually get a diagnosis before age 65, most often between their 20s and 40s. It happens two to three times more often in males.
What are the symptoms?
Shortness of breath, chest pain, fatigue, passing out (syncope), and heart palpitations. If the tumor spreads beyond the heart, anemia or weight loss may also occur.
What causes cardiac angiosarcoma?
There isn't a clear cause. People with certain DNA mutations, like the POT1 gene, may be more likely to get it. Prior radiation therapy to the chest to treat childhood cancer is another possibility.
What are the risk factors?
A history of radiation therapy for another cancer, exposure to certain cancer-causing chemicals, and lymphedema.
What complications can it cause?
Heart failure, blockage in your vena cava, arrhythmia (abnormal heart rhythm), pericardial effusion, cardiac tamponade, heart valve regurgitation (leak), and obstructive shock.
How is it diagnosed?
With a transthoracic echocardiogram, transesophageal echocardiogram, CT scan, heart MRI, PET scan, cardiac catheterization, and heart biopsy.
How is it treated?
Surgery can improve symptoms, and removing the whole tumor gives the best chance of surviving. Chemotherapy can ease symptoms and help some people live longer. Sometimes radiation can improve your situation.
What are the risks of surgery?
Complications may include bleeding, abnormal heart rhythm (arrhythmia), and heart dysfunction.
Does it spread before diagnosis?
Yes — it commonly spreads to the liver, lungs, and brain before diagnosis, and can also spread to bones and adrenal glands, which makes it hard to treat and survive.
What is the prognosis?
Very poor. Average survival for cardiac sarcoma is about 10 to 16 months; without surgery most people live 9 to 12 months; some estimates say as short as 5 months or less. About 14% of people are alive five years after diagnosis. Spread (metastasis) to other body parts is often fatal.
Can I lower my risk?
Limiting exposure to certain chemicals may help. If you have a genetic irregularity that raises your risk, talk to your provider — screenings may catch it early.
When should I go to the ER?
Get emergency help for shortness of breath, chest pain, or a fever or other signs of infection if you've had chemotherapy.
Related reading
Heart Tumor Removal Surgery: Approaches, Risks and Recovery explains heart tumor removal surgery.
This article is for general informational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions about a medical condition. Never disregard professional medical advice or delay seeking care because of something you have read here.
References
Cleveland Clinic. Cardiac Angiosarcoma: Symptoms & Outlook. Cleveland Clinic. Last updated January 2, 2024. https://my.clevelandclinic.org/health/diseases/cardiac-angiosarcoma
Center for Cancer Research, National Cancer Institute. Angiosarcoma. Cancer.gov. Posted February 27, 2019. https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-vascular-tumors/angiosarcoma
Elmusa E, et al. Primary Cardiac Angiosarcoma Presenting as Cardiac Tamponade. Cureus. 2022;14(9):e29033. PMID 36237796. https://doi.org/10.7759/cureus.29033
Guo S, et al. Primary Cardiac Angiosarcoma. Int Heart J. 2021;62(2):458-462. PMID 33731530. https://doi.org/10.1536/ihj.20-581
Kumari N, et al. Primary Cardiac Angiosarcoma: A Review. Cureus. 2023;15(7):e41947. PMID 37461430. https://doi.org/10.7759/cureus.41947
Saad AM, et al. Characteristics, survival and incidence rates and trends of primary cardiac malignancies in the United States. Cardiovasc Pathol. 2018;33:27-31. PMID 29414429. https://doi.org/10.1016/j.carpath.2017.12.001
Alahmadi MH, Rodriguez Ziccardi M, Tariq MA, Ahmed SW. Cardiac Tumors. Updated June 2, 2025. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2026 Jan-. PMID 30725829. https://www.ncbi.nlm.nih.gov/books/NBK537144/
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