Rhabdomyosarcoma

Rhabdomyosarcoma is a cancerous (malignant) tumor of the muscles that are attached to the bones. It can occur in many places in the body. The most common sites are the structures of the head and neck, the urogenital tract, and the arms or legs. Rhabdomyosarcoma is the most common soft tissue tumor in children. Causes The cause of rhabdomyosarcoma is unknown. It is a rare tumor with only several hundred new cases per year throughout the United States. Some children with certain birth defects are at an increased risk, and some families have a gene mutation that elevates risk. However, the great majority of children with rhabdomyosarcoma do not have any known risk factors. Symptoms The most common symptom is a mass that may or may not be painful. Other symptoms vary depending on location of the tumor. y y y y y y Tumors in the nose or throat may cause bleeding, congestion, swallowing problems, or neurological problems if they extend into the brain. Tumors around the eyes may cause bulging of the eye, problems with vision, swelling around the eye, or pain. Tumors in the ears, may cause pain, hearing loss, or swelling. Tumors in the vagina may be visibly protruding from the opening of the vagina. Bladder and vaginal tumors may cause lead to trouble starting to urinate or having a bowel movement, or poor control of urine. Muscle tumors may lead to a painful lump and are often thought to be an injury.

Exams and Tests Diagnosis of rhabdomyosarcoma is often delayed because of lack of symptoms, and because the tumor may appear at the same time as a recent injury. Early diagnosis is important because rhabdomyosarcoma is an aggressive tumor that spreads quickly. A complete physical exam should be done. Tests done to diagnose this condition may include: y y y y y y Biopsy of the tumor CT scan of the chest to look for spread of the tumor CT scan of the tumor site Bone marrow biopsy (may show the cancer has spread) Bone scan to look for spread of the tumor MRI scan of the tumor site

Spinal tap (lumbar puncture)

Treatment The precise treatment depends on the site and type of rhabdomyosarcoma. Because of its rarity, this tumor is best treated at a center with experience treating many patients with rhabdomyosarcoma. Either radiation or chemotherapy, or both, will be used before or after surgery. In general, surgery and radiation therapy are used to treat the primary site of the tumor. Chemotherapy is used to treat disease at all sites in the body. Chemotherapy is an essential part of treatment to prevent further spread of the cancer. Many different chemotherapy drugs are active against rhabdomyosarcoma. Some of these drugs include: y y y y y Dacarbazine Doxorubicin Epirubicin Gemcitabine Ifosfamide

Support Groups The stress of illness can often be helped by joining a support group where members share common experiences and problems. See cancer - support group. Outlook (Prognosis) While aggressive treatment is usually necessary, most children with rhabdomyosarcoma will achieve long-term survival. Cure depends on the specific type of tumor, its location, and the amount that has spread. Possible Complications y y y Complications from chemotherapy Location in which surgery is not possible Metastasis

When to Contact a Medical Professional Call your health care provider if your child has symptoms of rhabdomyosarcoma. Alternative Names Soft tissue sarcoma; Alveolar rhabdomyosarcoma; Embryonal rhabdomyosarcoma; Sarcoma botryoides

Rhabdomyosarcoma, pleomorphic - Hand

Rhabdomyosarcoma, alveolar Thigh

ORBIT

Rhabdomyosarcoma is a fast-growing, highly malignant tumor which accounts for over half of the
soft tissue sarcomas in children. Less frequently, other soft tissue sarcomas are found in children: fibrosarcoma, mesenchymoma, synovial sarcoma, and liposarcoma. Rhabdomyosarcoma often causes a noticeable lump on a child's body. If the tumor is located internally, the symptoms depend on its location. For example, tumors in the nasal passage may put pressure on the eustachian tubes; a bladder tumor can cause trouble urinating; an orbital tumor may cause the eye to protrude.

About Rhabdomyosarcoma
Rhabdomyosarcoma tumors arise from a cell called a "rhabdomyoblast", which is a primitive muscle cell. 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:

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head and neck; around the eyes -- 35-40% genitourinary tract -- 20% extremities -- 15-20% trunk (chest and lungs) -- 10-15%

Depending on the "histology" of the cells (how they look under a microscope), the tumors are classified as one of the following: Embryonal rhabdomyosarcoma. Most common type, usually found in children under 15 and in the head and neck region and genitourinary tract. Botryoid type. A variant of the embryonal type; the tumor arises as a grape-like lesion in mucosallined hollow organs such as the vagina and urinary bladder. Alveolar type. A more aggressive tumor which usually involves the muscles of the extremities or trunk. Pleomorphic type. 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. The Intergroup Rhabdomyosarcoma Study Group has defined a set of guidelines, which assign the tumor to groups 1-4 depending on the extent of the disease. For more information, see the PDQ on the staging of rhabdomyosarcoma.

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 y y y y y
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 in 1-5 age group. Overall, 50% of the children diagnosed with rhabdomyosarcoma survive 5 years.

Ped-Onc Resources for Rhabdomyosarcoma

The following ped-onc resource lists have appropriate sections for parents of children with rhabdomyosarcoma:

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childhood cancer e-mail lists - the ACOR rhabdomyosarcoma list (Rhabdo-kids) and the general childhood cancer list, ped-onc, are both appropriate support organizations books and printed materials - especially the book Childhood Cancer, A Parents's Guide to Solid Tumor Cancers, by Honna Janes-Hodder and Nancy Keene. young people with rhabdomyosarcoma - personal home pages