PEComa | Sarcoma UK
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PEComa is a very rare type of soft tissue sarcoma that develops from the cells lining the blood vessels.

PEComa, also known as perivascular epithelioid cell tumour, is a very rare sarcoma that can occur anywhere throughout the body. But, it most often occurs in the uterus, skin, liver and gut.

PEComa is most commonly associated with young to middle-aged adults. It is also far more common in women.

The cause of PEComa is unknown, but researchers think that PEComa is caused by errors in genes called TSC1 and TSC2. Researchers are still trying to find out why these errors happen and whether this causes PEComa. In some cases, people with a disease called tuberous sclerosis may have a greater risk of developing PEComa.

Signs and symptoms

Symptoms of PEComa 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 PEComa:

  • A lump or swelling in the soft tissue of the body under the skin
  • This lump is often slow-growing and can be painful or painless, depending on where it is
  • Bleeding from the vagina if the PEComa is within the reproductive system of females
  • PEComa within the gut can result in stomach pain, blood in your poo, weight loss and constipation


A specialist doctor will diagnose PEComa through a series of tests. These may include:

  • Physical examination – looking at and feeling any lump
  • A scan – taking pictures of the inside of the body using scans such as CT, PET or MRI 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 PEComa and whether it contains the TSC1 and TSC2 errors

To read more about these scans and tests, click here



  • The first treatment method for PEComa 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 PEComa, researchers have been learning more about treatments known as “targeted therapies” and “immunotherapies”

Targeted therapy is a treatment that targets specific characteristics within cancer cells in order 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.

  • There is a targeted therapy that can be used for treating PEComa. This is called nab-sirolimus and can be used if your tumour cannot be removed safely or if it has spread to other parts of the body
  • 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

Clinical Trials

For all sarcoma clinical trials based in the UK that are currently recruiting, you can check 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. The follow-ups will usually include:

  • A chance to discuss symptoms
  • An examination to look for any signs of PEComa 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

Living with

PEComa 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 PEComa 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 PEComa tumour. In PEComa patients, these secondary cancers may appear in the lungs, which is why a chest x-ray is taken at follow-up appointments.

Secondary cancers may also appear in the liver, lymph nodes and bones. Treatment for secondary cancer may involve surgery, radiotherapy or other therapy as appropriate; your treatment will be assessed on an individual basis.

If you have any questions or if you need to talk to someone, our Support Line team are here for you.


More information –

A scientific review on the current management of PEComa

Last reviewed: 3 January 2023

Next review due: 3 January 2026


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