Rhabdomyosarcoma is cancer of the connective tissue that is a fast growing tumor. It is most commonly found in the head and neck. This cancer can also occur in the legs, arms, abdomen, bladder, urinary tract and sex organs. Rhabdomyosarcoma most commonly occurs in children between the ages of one and five. This disease has been diagnosed in adolescents aged 15 to 19, but rarely. When diagnosed in adults, this cancer is usually present in the large muscles of the legs and arms. Only about 350 children are diagnosed with this disease per year in the United States. Cure rate for this cancer is 70 to 75 percent.
Symptoms of Rhabdomyosarcoma depend on the location and size of the tumor. The first sign is a hard lump or mass that persists or grows. There may be some pain associated with the mass. Most adult patients have pain deep in the muscle of the arm or leg.
Diagnosis is made by a biopsy in which a surgeon removes a small piece of tissue from the lump or mass. A pathologist will examine the cells to determine if it is cancerous. Rhabdomyosarcoma develop from embryonic or “pre” muscle cells that grow out of control instead of growing into striated muscle cells. The most prominent sites for tumors are the head and neck, especially around the eyes (35-40%), the genitourinary tract (20%), the trunk, including chest and lungs (10-15%) and the arms and legs (15-20%).
Tumors are classified by cell type:
Embryonal Rhabdomyosarcoma is the most common found in the genitourinary tract, head and neck in children under 15 years of age.
Botryoid type produces a lesion shaped like a grape in hollow organs such as the bladder and the vagina.
Alveolar type involves the muscles of the arms, legs or trunk. Alveolar Rhabdomyosarcoma is a fast growing tumor.
The most treatable form of Rhabdomyosarcoma is the embryonal type. Prognosis depends on the location and stage of the tumor. Genitourinary tract and around the eyes generally have a better prognosis than tumors that form elsewhere on the body. Tumors are assigned into stages one through four based on the United States National Cancer Institute. Stage 1 is defined as localized Rhabdomyosarcoma on the head, neck, orbit or the genitourinary region. These regions do not include the biliary (ducts of the liver)/prostate sites or the epidural/subdural spaces surrounding the brain and spinal cord. Stage 2 is defined as localized disease of any primary sites that aren’t listed in stage 1. Tumors are 5 cm or smaller and no cancer is present in lymph nodes in this stage. Stage 3 is defined as cancer being present in any of the other primary sites, the tumor is greater than 5 cm and there is cancer in the lymph nodes near the tumor. Stage 4 is defined as Rhabdomyosarcoma that has metastasized or spread to other areas of the body from the original tumor site.
Treatment for Rhabdomyosarcoma depends on the location of the tumor, the stage of the tumor’s development, age of the patient, health of the patient and the type of Rhabdomyosarcoma.
Treatment options must be discussed with an oncologist that specializes in Rhabdomyosarcoma. Treatment includes chemotherapy, radiation therapy and surgery for this disease. Radiation therapy is usually combined with surgery – either before surgery or before and after surgery or simply after surgery. Radiation therapy is used to shrink tumors prior to surgery and to kill any residual cancer cells after surgery. Chemotherapy involves a combination of cancer cell killing medications and is usually applied after surgery.
Radiation therapy can also include intraoperative radiation therapy, also known as IORT. This procedure involves the placement of hallow rods while surgery is being performed. Then after surgery brachytherapy seeds – which are radio active can be inserted into the rods. Tumors can be treated using very exact technology – such as intensity modulated radiation or 3D conformal radiation.
A cure for Rhabdomyosarcoma is based on five factors. These factors are the age of the patient, where the tumor is in the patient, the size of the tumor at initial diagnosis, they type of cells that make up the tumor and if the cancer has spread to other places in the body.
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