This information is about intimal sarcoma. It includes the signs and symptoms, how it’s diagnosed, and how it’s treated.
What is intimal sarcoma?
Intimal sarcoma is a very rare sarcoma cancer. It usually grows inside large blood vessels. It more commonly grows in the pulmonary artery, which is the large artery that carries blood from the heart to the lungs.
It can also grow in the heart or the aorta. The aorta is the main blood vessel that carries blood from the heart to the rest of the body.
Who gets intimal sarcoma?
Intimal sarcoma is most common in adults aged 40 to 65.
- Intimal sarcoma of the pulmonary artery and heart is slightly more common in females.
- Intimal sarcoma in the aorta is more common in males.
What causes intimal sarcoma?
Researchers think intimal sarcoma starts when there are too many copies of certain genes in the cells. This is called gene amplification.
Researchers are still trying to find out why this happens and whether gene amplification is a cause.
Symptoms of intimal sarcoma
Symptoms of intimal sarcoma can vary depending on the size and location of the tumour. You may have all, some, or none of these symptoms before you are diagnosed.
If the tumour is in the pulmonary artery, you may have:
- shortness of breath
- pain in the chest or back
- a cough that does not go away
- coughing up blood
- light-headedness and fainting
If the tumour is in the heart or aorta, you may have:
- pain in the arms or legs
- problems with your heart rhythm
- pain in the chest, back or stomach
- tiredness
- shortness of breath
- high blood pressure.
As intimal sarcoma is very rare, it is commonly misdiagnosed as conditions of the heart and blood vessels. For example, it can be wrongly diagnosed as blood clots, embolisms, or atherosclerosis.
Diagnosing intimal sarcoma
A specialist doctor will diagnose intimal sarcoma through a series of tests. These may include:
- a physical examination
- a scan – taking pictures of the inside of the body using scans such as CT, PET, or MRI
- a biopsy – taking and testing a sample of the tumour.
Find out more about the different types of scans and tests.
Treating intimal sarcoma
Surgery
The most common treatment for intimal sarcoma is surgery. A surgeon will remove the tumour and take out an area of healthy tissue surrounding it. This is called taking a margin. This is not always possible if the tumour in the heart or large blood vessels.
Radiotherapy
Radiotherapy is a treatment that uses high-energy radiation beams to destroy cancer cells. It can be used before or after surgery.
- When used before surgery, radiotherapy can make the tumour smaller so that it’s easier to remove.
- When used after surgery, radiotherapy can kill off any remaining cancer cells that could not be removed by surgery.
Chemotherapy
Chemotherapy is a treatment that uses anti-cancer drugs to destroy cancer cells. It is sometimes used for people who have a high risk of intimal sarcoma coming back, or if the cancer has spread (metastasis).
Targeted and experimental therapies
Researchers have found that some targeted therapies and immunotherapies worked well in clinical trials. Researchers need to do more studies before doctors can use these treatments for intimal sarcoma.
- Targeted therapy is a treatment that targets specific features within cancer cells to stop them from growing and spreading.
- Immunotherapy is a treatment that helps the person’s own immune system to fight and kill cancer cells.
Your doctor or nurse can give you information about clinical trials using targeted therapy or immunotherapy where available.
You can also search for clinical trials on our clinical trials hub.
After treatment
After treatment, you will have regular follow-up appointments for several years. You should receive a follow-up schedule from your sarcoma clinical nurse specialist.
Follow-up appointments
Follow-up usually includes:
- a chance to discuss symptoms
- an examination to look for any signs of intimal sarcoma returning
- scans such as CT, PET or MRI scans
- a chest X-ray to check for cancer in the lungs.
Recurrence
Intimal sarcoma can reappear in the same area after the treatment of a previous tumour. This is called a local recurrence.
Tumours can also reappear in other parts of the body. This is called metastasis or secondary cancer. Secondary tumours might appear in the lungs, which is why you will have chest X-rays during follow-up care.
If the cancer does reappear, it is important to get treated as quickly as possible. If you are worried about your cancer returning, contact your doctor or nurse. They may offer you an earlier follow-up appointment to check your symptoms.
Living with intimal sarcoma
Living with intimal sarcoma can be hard. We have support to help you with your mental health, physical health, and we also have information about financial support. No matter what you’re worried about, we’re here to support you.
If you have any more questions about intimal sarcoma, or if you need to talk to someone, our Support Line team are here for you.
More support
Call the Support Line
The Sarcoma UK Support Line is here for every person affected by sarcoma. Get in touch for friendly, expert, confidential and free advice on anything to do with sarcoma.
Join a support group
There are a number of sarcoma support groups across the UK. We have more information about support groups available online and in your local area.
Support from other charities
- Maggie’s: Maggie’s is a charity that offers advice and support for adults and young people living with cancer.
- Penny Brohn:Penny Brohn has a lot of support for people who have just been diagnosed or are living with cancer. This includes resources, online sessions, and counselling.
- Shine Cancer Support: Shine Cancer Supportis a charity connecting people with cancer experience through online programmes and in-person support groups. They support people in their 20s, 30s, and 40s.
- Macmillan:Macmillan Cancer Support has a lot of information about cancer diagnosis, treatment, and after treatment.
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Last reviewed August 2026 | Next review due August 2029
All references are available upon request. Please email info@sarcoma.org.uk
