There are an average of 36 cases of ERMS diagnosed every year in England.

ERMS makes up around 32% of all rhabdomyosarcoma diagnoses.

ERMS tends to affect slightly more females than males.

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

Symptoms of ERMS can vary depending on the size and location of your tumour. The main symptom of ERMS is a lump, lesion or swelling in the soft tissue of the body under the skin, often in the head or neck. This lump is often fast-growing, but usually 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 genitourinary system may cause symptoms such as:
  • Lumps or swelling in the genitals, commonly on the scrotum
  • Trouble peeing
  • Blood in the pee

• Yellowing of the skin and the whites of the eyes, known as jaundice

• Surgery
  • The first treatment method for ERMS 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.

• 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 ERMS. 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 ERMS. 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 ERMS 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.

ERMS 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 ERMS 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 ERMS tumour. In ERMS 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.