Pleomorphic rhabdomyosarcoma | Sarcoma UK
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Pleomorphic rhabdomyosarcoma

Pleomorphic rhabdomyosarcoma, also known as “PRMS”, is a type of soft tissue sarcoma.

It develops from the cells in skeletal muscle, usually in adults.

It can occur anywhere in the body. But, it most often occurs in the legs. It also often occurs in the chest, abdomen, arms, and the head and neck area.

Rhabdomyosarcoma is very rare in older adults. The most common subtypes of rhabdomyosarcoma are embryonal rhabdomyosarcoma and alveolar rhabdomyosarcoma. These two subtypes usually occur in children, teenagers and young adults. Of the rare cases of rhabdomyosarcoma that do occur in adults, PRMS is the most common subtype.

PRMS is most commonly associated with older adults aged 50 and over.

The cause of most PRMS is unknown. Researchers are still trying to learn more about the causes of PRMS.


There are an average of 14 cases of PRMS diagnosed every year in England.

PRMS makes up around 13% of all rhabdomyosarcoma diagnoses.

For more information on these statistics, you can visit our soft tissue sarcoma data hub.

Signs and symptoms

Symptoms of PRMS can vary depending on the size and location of your tumour. The main symptom of PRMS is a lump, lesion or swelling in the soft tissue of the body under the skin, often in the arms and legs. This lump is often fast-growing, and can be painful or painless.

  • A lesion in the head or neck can also cause other symptoms, such as:
    • Bulging of the eyes
    • Double vision
    • Swelling of the sinuses
    • Trouble swallowing or hearing
  • A lesion in the abdomen may cause symptoms such as:
    • Constipation
    • Abdominal pain, swelling, or bloating
    • Blood in the pee or poo


A specialist doctor will diagnose PRMS 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, MRI, PET, x-ray, or ultrasound 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 PRMS.
  • Bone scans and bone marrow biopsies – PRMS sometimes spreads to the bone marrow. Bone scans and bone marrow biopsies can test for this.

To read more about these scans and tests, click here.


  • Surgery
    • The first treatment method for PRMS 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 the cancer has been removed.
    • PRMS mostly affects the arms and legs. Surgeons will do limb-sparing surgery so that the limb can continue to work well.
    • Very rarely, the cancer can spread throughout the limb. In this case, the surgeon may have to perform a partial or full amputation to stop the cancer.
  • Radiotherapy
    • 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. This is so that it can be more easily removed.
      • When used after surgery, radiotherapy aims to kill off any remaining cancer cells that were not removed by surgery.
  • Chemotherapy
    • This treatment uses anti-cancer drugs to destroy cancer cells. It’s sometimes used in patients that have a high risk of the cancer coming back. It’s also sometimes used if the cancer has spread to other parts of the body, also known as “metastasis”.
  • Targeted and experimental therapies
    • 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. It does this to stop them from growing and spreading.
      • Immunotherapy is a treatment that helps a person’s immune system fight and kill cancer cells.
    • Some targeted therapies and immunotherapies have worked well in clinical trials of PRMS. We need more research and trials before these treatments can be approved.
  • Clinical trials
    • Your doctor may offer you an opportunity to take part in a trial. The trial might 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 taking part in a clinical trial.
    • There are trials currently recruiting patients with PRMS. For more information, please follow this link.

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 PRMS returning. This may include scans such as CT or MRI scans.
  • A chest x-ray and bone scans to rule out any secondary cancers occurring in the lungs or bone marrow.

Living with

PRMS 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 PRMS 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 PRMS tumour. In PRMS 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 bone marrow and lymph nodes. 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 clinical study of PRMS

Last reviewed: 6 March 2023

Next review due: 6 March 2026


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