Rhabdomyosarcoma
What is Rhabdomyosarcoma?
About Rhabdomyosarcoma
Treatment
Statistics
More Information
What is Rhabdomyosarcoma?
Rhabdomyosarcoma is a type of sarcoma, meaning a cancer of the bone, soft tissues, or connective tissue (e.g., tendon or cartilage). Rhabdomyosarcoma is a fast-growing, highly malignant tumor which begins growing in the striated muscle of soft tissues. It can occur anywhere in the body, often causing a noticeable lump. However, if located internally, symptoms are dependent on location. For example, nasal passage tumors may put pressure on the Eustachian tubes; a bladder tumor may cause trouble urinating, and orbital tumors may cause eye protrusion.
About Rhabdomyosarcoma
Rhabdomyosarcoma tumors arise from a primitve muscle cell called a "rhabdomyoblast." Instead of differentiating into striated muscle cells, the rhabdomyoblasts grow out of control. Since this type of muscle is located throughout the body, the tumors can appear at numerous locations. The four major sites in which rhabdomyosarcoma is found are:
- Head and neck; around the eyes — 35-40%
- Genitourinary tract — 20%
- Extremities — 15-20%
- Trunk (chest and lungs) — 10-15%
Embryonal: Most common type, usually found in children under 15 and in the head and neck region and genitourinary tract.
Botryoid: A variant of the embryonal type; the tumor arises as a grape-like lesion in mucosal-lined hollow organs such as the vagina and urinary bladder.
Alveolar: A more aggressive tumor which usually involves the muscles of the extremities or trunk.
Pleomorphic: Usually seen in adults and arises in muscles of the extremities.
Embryonal rhabdomyosarcoma is considered the most treatable form of the disease. The prognosis is also affected by the location of the primary tumor. Orbital and genitourinary track rhabdomyosarcomas have a better prognosis than do tumors which originate in the head and neck, extremity, pelvic, and trunk locations.
Prognosis also depends on the stage of the tumor.
Treatment
Rhabdomyosarcoma is treated by a combination of surgery, chemotherapy, and radiation.
Surgery. Resection (removal) of the primary tumor. If necessary after chemotherapy or radiation has shrunk the tumor.
Chemotherapy. The following chemotherapy agents are commonly used: vincristine, cyclophosphamide, dactinomycin, adriamycin, ifosfamide, VP-16.
Radiation. External beam radiation is used in some cases of rhabdomyosarcoma.
Rhabdomyosarcoma clinical trials, NCI search form
Statistics
- Rhabdomyosarcoma accounts for 5-8% of childhood cancers
- 70% of all Rhabdomyosarcoma cases diagnosed in the first ten years of life
- Usually affects children, the ages of 2 to 6 and 15 to 19
- The peak incidence occurs in 1-5 age group
- Overall, 50% of the children diagnosed with Rhabdomyosarcoma survive 5 years
For more information about Rhabdomyosarcoma, click on any of the links below:
WebMDHealth
Pediatric Oncology Resource Center
National Cancer Institute