Merck Manual

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Kee Kiat Yeo

, MD, Harvard Medical School

Reviewed/Revised May 2023
Topic Resources

Rhabdomyosarcoma is a fast-growing cancer that can develop in soft tissues (such as muscle) almost anywhere in the body.

  • Rhabdomyosarcomas can develop anywhere, but the head and neck, genital or urinary tract, and limbs are the most common locations.

  • Symptoms depend on where rhabdomyosarcomas develop.

  • Diagnosis involves imaging tests and a biopsy.

  • Treatment consists of chemotherapy, surgery, and sometimes radiation therapy.

About 400 to 500 cases of rhabdomyosarcoma occur in children in the United States each year. This cancer accounts for 3% of all cancers in children. Two thirds of cases of this cancer are diagnosed in children younger than 7 years of age. Rhabdomyosarcomas occur mostly in children.

This cancer develops from cells that would normally develop into muscle cells. The cause of rhabdomyosarcoma is unknown.

Although rhabdomyosarcoma can occur almost anywhere, it tends to occur most often in the following:

  • Head and neck (in about 35% of cases): Most common among school-aged children

  • Genital or urinary tract (in about 25% of cases), usually in the bladder, prostate, or vagina: Typically occurring in infants and toddlers

  • Limbs (in about 20% of cases): Most common among adolescents

  • Trunk or other miscellaneous sites (in about 20% of cases)

Rhabdomyosarcoma can spread (metastasize) to other parts of the body. But it is usually diagnosed before the cancer has spread. About 15 to 25% of children are diagnosed after the cancer has spread. In these children, the cancer is most commonly located in the lungs. Bones, bone marrow, and the lymph nodes are other locations to which the cancer may spread.

Symptoms of Rhabdomyosarcoma

In most children, the first symptom of the cancer is a firm lump or problems related to an organ affected by the cancer, such as the following:

  • Eyes: Tearing, eye pain, or a bulging eye

  • Nose and throat: Nasal congestion, a change in the voice, or a nasal discharge that contains mucus and pus

  • Genital or urinary tract: Abdominal pain, a lump in the abdomen that can be felt, difficulty urinating, and blood in the urine

  • Vagina: Discharge that contains mucus and blood or a polyp-like mass that sticks out of the vagina

  • Limbs: Firm lumps on the arms or legs

Limb cancers frequently spread, especially to the lungs, bone marrow, and lymph nodes. Usually, this spread does not cause symptoms.

Diagnosis of Rhabdomyosarcoma

  • Computed tomography (CT) or magnetic resonance imaging (MRI)

  • Biopsy or removal of tumor

The diagnosis of rhabdomyosarcoma is confirmed by taking a sample from the lump and examining it under a microscope (biopsy). Sometimes the entire lump is removed surgically.

Treatment of Rhabdomyosarcoma

  • Chemotherapy and surgery

  • Sometimes radiation therapy

All children are treated with chemotherapy. The most common chemotherapy drugs used are vincristine, dactinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide. Topotecan and irinotecan are other drugs that may also be used.

When surgery is done, the entire cancer is removed if possible.

Radiation therapy is typically used if some cancer remains after surgery or if the cancer is considered intermediate risk or high risk.

Prognosis for Rhabdomyosarcoma

The prognosis is based on several factors:

  • Where the cancer is located

  • How much of the cancer can be removed

  • Whether it has spread

  • How old the child is

  • What the cancer cells and tissue look like when examined under a microscope

The prognosis is worse for children who are under 1 year of age or who are over 10 years of age.

Children are considered low risk, intermediate risk, or high risk depending on their combination of factors. More than 90% of children who are considered low risk survive, but less than 50% of children who are considered high risk survive.

More Information

The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.

  • American Cancer Society: If Your Child Is Diagnosed With Cancer: A resource for parents and loved ones of a child who has cancer that provides information about how to cope with some of the problems and questions that come up just after a child is diagnosed

Drugs Mentioned In This Article

Generic Name Select Brand Names
Oncovin, Vincasar PFS
Cyclophosphamide, Cytoxan, Neosar
Adriamycin, Adriamycin PFS, Adriamycin RDF, Rubex
Etopophos, Toposar, VePesid
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