Intimal sarcoma is a very rare type of soft tissue sarcoma that develops from cells in the large blood vessels of the body and sometimes in the heart.
Intimal sarcoma is a very rare sarcoma that occurs in the large blood vessels that carry blood around the body. It most commonly occurs in the large artery that carries blood from the heart to the lungs, called the pulmonary artery. It can also occur in the heart itself or in a large artery that carries blood from the heart to the rest of the body, called the aorta.
Intimal sarcoma is most commonly associated with middle-aged adults, with the most common age at diagnosis being between 48–62 years old.
Intimal sarcoma that occurs in the pulmonary artery and heart are slightly more common in females, while intimal sarcomas in the aorta are thought to be more common in males.
Intimal sarcoma is thought to be caused when there are too many copies of certain genes in the cells. This is most commonly caused by something called gene amplification. Researchers are still trying to find out why this happens and whether this causes intimal sarcoma.
Signs and symptoms
Symptoms of intimal sarcoma can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with intimal sarcoma.
If the intimal sarcoma is in the pulmonary artery, you may experience:
- Shortness of breath
- Pain in the chest or back
- Continuous cough, which may contain blood
- Light-headedness and fainting
- Feeling ill or tired
If the intimal sarcoma is in the heart or aorta, you may experience:
- Pain in the arms or legs
- Problems with your heart rhythm
- Pain in the chest, back or stomach
- Feeling ill or tired
- Shortness of breath
- High blood pressure
As intimal sarcoma is very rare, it is frequently misdiagnosed as more common conditions that affect the heart and blood vessels, such as blood clots, embolisms, or atherosclerosis.
Any signs and symptoms related to the heart or major blood vessels of the body should be looked at by a doctor immediately.
A specialist doctor will diagnose intimal sarcoma through a series of tests. These may include:
- Physical examination – looking for any signs and symptoms of intimal sarcoma
- A scan – taking pictures of the inside of the body using scans such as CT, MRI or PET scans
- A biopsy – taking and testing a sample of the tumour. Using this biopsy, a doctor will look to see if the biopsy looks like intimal sarcoma and whether it contains the gene errors
To read more about these scans and tests, click here.
- The first treatment method for intimal sarcoma is surgery. The surgeon will remove the tumour and will aim to take out an area of normal tissue too – this is known as “taking a margin”. This is to make sure that all of the cancer has been removed
- This treatment uses high-energy radiation to destroy cancer cells
- It can be used either before or after surgery
- When used before surgery, radiotherapy aims to make the tumour smaller so that it can be more easily removed
- When used after surgery, radiotherapy aims to kill off any remaining cancer cells that have not been removed by surgery
- This treatment uses anti-cancer drugs to destroy cancer cells. It is sometimes used in patients that have a high risk of the cancer coming back or if it has spread to other parts of the body, also known as “metastasis”.
Targeted and experimental therapies
- In intimal sarcoma, researchers have been learning more about treatments known as “targeted therapies” and “immunotherapies”
- Targeted therapy: a treatment that targets specific characteristics within cancer cells in order to stop them from growing and spreading
- Immunotherapy: a treatment that helps the person’s own immune system to fight and kill cancer cells
- Researchers have found that some targeted therapies and immunotherapies worked well in clinical trials of intimal sarcoma. But, more research and trials are needed before these treatments are approved
- You may be offered an opportunity to take part in a trial to investigate new diagnosis methods, drugs or treatments. Some studies also look at the care and well-being of patients. Your doctor or nurse can give you more information on opportunities for you to take part in a clinical trial
- There are trials currently recruiting patients with intimal sarcoma. For more information, please follow this link
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. The follow-ups will usually include:
- A chance to discuss symptoms
- An examination to look for any signs of intimal sarcoma returning. This may include scans such as CT, PET or MRI scans
- A chest x-ray to rule out any secondary cancers occurring in the lungs
Intimal sarcoma can reappear in the same area after the treatment of a previous tumour; this is called a “local recurrence”.
If the cancer does reappear, it is important to get treated as quickly as possible. This could involve further surgery and/or radiotherapy; your treatment will be assessed on an individual basis. It is useful to check for recurrences yourself through self-examination: your doctor or sarcoma clinical nurse specialist can tell you what to look for.
If you are worried about your cancer returning, please contact your doctor or nurse. They may decide to bring forward the date of your follow-up appointment to investigate your concerns.
What if my cancer spreads to another part of my body?
A recurrence of intimal sarcoma may be accompanied by cancer in other parts of the body. This is called “metastasis” or “secondary cancer”. Some people are diagnosed with sarcoma because their metastases have been discovered before their primary intimal sarcoma tumour. In intimal sarcoma patients, these secondary cancers may appear in the lungs, which is why a chest x-ray is taken at follow-up appointments.
Secondary cancers of intimal sarcoma are rare outside of the lungs, but have been reported in the lymph nodes, kidneys, brain, bones and liver. Treatment for secondary cancer may involve surgery, radiotherapy or other therapy as appropriate; your treatment will be assessed on an individual basis.
Last reviewed: 2 February 2023
Next review due: 2 February 2026