Rhabdomyosarcoma: Causes, Symptoms, Treatment

Rhabdomyosarcoma is a type of cancer that affects muscle tissue. It’s most common in children and adolescents.

The disease starts in the mesenchymal cells, which are cells that turn into muscle. With rhabdomyosarcoma, the cells change and grow out of control, forming one or more tumors.

Rhabdomyosarcoma

Rhabdomyosarcoma can occur anywhere in the body, but it’s most common in the following places:

• Arms and legs.
• Head and neck.
• Urinary and reproductive organs.

How common is this type of sarcoma?

Rhabdomyosarcoma is rare. Only about 500 people are diagnosed each year in the United States. Most of them are children or adolescents.

Are there different types of rhabdomyosarcoma?

The types of rhabdomyosarcoma may include:

• Embryonal rhabdomyosarcoma: This is the most common type. It usually occurs in the head and neck or the reproductive and urinary organs.
• Botryoid rhabdomyosarcoma: This is a subtype of embryonal rhabdomyosarcoma. It’s found most often in hollow organs (e.g., bladder, vagina).
• Spindle rhabdomyosarcoma: This is also a subtype of embryonal rhabdomyosarcoma. It’s usually found around a boy’s testicles.
• Alveolar rhabdomyosarcoma: This is an aggressive type, usually found in the arms, legs or torso.
• Pleomorphic and undifferentiated rhabdomyosarcoma: These types are rare in children. When they do occur, it’s usually in the arms, legs or torso.

What causes rhabdomyosarcoma?

Scientists aren’t sure what causes rhabdomyosarcoma. They’re studying how changes to DNA might cause cells to become cancerous.

What are the symptoms of rhabdomyosarcoma?

Symptoms of rhabdomyosarcoma depend on where the cancer is in the body. But common signs include:

• Bleeding from the nose, throat, vagina or rectum (where bowel movements come from).
• Blood in urine (pee).
• Eyes that cross or bulge.
• Headaches.
• Lump that gets bigger or doesn’t go away.
• Trouble peeing or pooping.

How is rhabdomyosarcoma diagnosed?

To diagnose rhabdomyosarcoma, a healthcare provider will perform:

• Health history to learn about symptoms and risk factors for the disease.
• Physical exam to look for any signs of disease, such as a lump.
• Tests that take pictures inside the body, such as X-ray, MRI, CT scan.
• Tests that look for cancerous cells in the body, such as a PET scan (positron emission tomography) or bone scan.

A healthcare provider might also order some tests to remove a sample of tissue from the body for testing. Examples include:

• Biopsy: Biopsy is the only way to confirm cancer. It can be done several different ways, depending on where cancer is suspected. Biopsy takes a small amount of tissue from the tumor for examination under a microscope.
• Bone marrow biopsy: Bone marrow biopsy uses a needle to remove a small amount of bone, blood and bone marrow, usually from one or both hip bones.
• Lumbar puncture: Also called spinal tap, this test uses a needle to take fluid from the spine for testing.

Why is rhabdomyosarcoma staged?

Test results will help your child’s healthcare team stage the disease. Staging defines:

• Where the cancer is.
• If or where it has spread.
• How large the tumor is.
• What the outlook is (prognosis).
• What kinds of treatments are best.

There are many ways to stage rhabdomyosarcoma. Talk to your healthcare provider about what stage your child has and what that means.

How is rhabdomyosarcoma treated?

Your child’s healthcare team will recommend a combination of treatments depending on:

• Stage.
• Type of rhabdomyosarcoma.
• Possible side effects.
• The person’s overall health and preferences.

The major types of treatment are:

• Chemotherapy: With chemotherapy, medications are injected into the bloodstream. The drugs kill cancer cells or stop them from dividing and spreading. Most children with rhabdomyosarcoma receive chemotherapy to limit the chance that the cancer will come back. Studies to test new drugs and combinations are ongoing.
• Surgery: A surgeon will cut out part or all of the tumor and some tissue around it. Surgical removal, also called excision, is used in almost all cases of rhabdomyosarcoma.
• Radiation: Radiation therapy uses beams of intense energy (usually high-energy X-rays) to kill cancer cells or slow their growth.
• Palliative care: Palliative care, also called supportive care, treats a person’s symptoms and the side effects of medications. It also may involve treatments for emotional, mental and social needs. Examples include medication, nutrition, therapy and relaxation.

Because rhabdomyosarcoma is so rare, many people with the disease are treated as part of clinical trials. Clinical trials are studies that test the newest, most promising treatments. Ask your healthcare team about clinical trials and whether your child is eligible to join.

Do treatments cure rhabdomyosarcoma?

Sometimes treatments can cure rhabdomyosarcoma. This is called remission, which means that cancer can no longer be detected.

In many cases, remission is permanent, but rhabdomyosarcoma can come back. This is called recurrence. Recurrence can happen in the same place in the body or a different part.

Your healthcare team will classify the disease into a risk group, which is a way to predict the chance that rhabdomyosarcoma will recur. The risk group (low, intermediate or high) will also help the healthcare team determine the best treatment or combination of treatments. If rhabdomyosarcoma recurs, your child’s healthcare team will do more tests and recommend more treatments.

How can I reduce the risk of my child getting rhabdomyosarcoma?

It isn’t clear who gets childhood rhabdomyosarcoma or why. There is currently no way to prevent it or to reduce the risk.

Are there other conditions that put my child at higher risk for rhabdomyosarcoma?

People with certain genetic disorders have an increased risk for rhabdomyosarcoma:

• Beckwith-Wiedemann syndrome.
• Cardiofaciocutaneous syndrome.
• Costello syndrome.
• Dicer1 syndrome.
• Li-Fraumeni syndrome.
• Neurofibromatosis type 1.
• Noonan syndrome.

What is the outlook for people with rhabdomyosarcoma?

About 70% of people with rhabdomyosarcoma survive five years or longer. But the outlook varies quite a lot depending on several factors, including:

• Age.
• Location and size of the original tumor.
• Risk group (low, intermediate or high).
• Type of rhabdomyosarcoma.
• Stage of disease.
• Success of surgery, chemotherapy or radiation to treat the tumor.
• Whether the cancer spread to other parts of the body.

Your healthcare provider can tell you more about your child’s prognosis.

What can I ask my child’s healthcare team about rhabdomyosarcoma?

If your child has been diagnosed with rhabdomyosarcoma, consider asking your healthcare team the following questions:

• What type of rhabdomyosarcoma does my child have?
• What stage is it, and why?
• What treatments do you recommend?
• What are the risks of those treatments and their side effects?
• What is my child’s prognosis?
• Should we see a specialist?
• Are we eligible for any clinical trials?
• What supportive care can you offer to help us?

How can my family cope with a diagnosis of rhabdomyosarcoma?

It’s normal to experience stress, fear and anxiety when you or a loved one is diagnosed with cancer. Talk to your healthcare team about ways to cope. Also consider:

• Counseling, or talk therapy.
• Relaxation techniques, such as meditation.
• Research so that you understand medical terms and treatment options.
• Stress reduction, such as exercise and time with friends.
• Support groups (in person or online) to connect with other families facing cancer.