Information about Childhood Rhabdomyosarcoma WHAT IS RHABDOMYOSARCOMA? Rhabdomyosarcoma is cancerous tumor involving muscle cells. Normally, cells grow and divide to form new cells as our body needs them. When cells grow old, they die and new ones take their place. However, there are times when this orderly process goes wrong. New cells form even when the body does not need them or old cells do not die when they should. Rhabdomyosarcoma occurs more often in children compared to adults. Although it can occur in any muscle containing area in the body, it most commonly involves muscles around the eye and head and neck area, followed by the bladder and prostate, trunk and arms and legs. WHAT ARE THE TYPES OF RHABDOMYOSARCOMA AND WHAT CAUSES IT? There are several types of rhabdomyosarcoma depending on its appearance under microscopic examination. The exact cause of rhabdomyosarcoma is not known although certain factors have been identified as being associated with an increased risk of developing the disease. This includes some very rare inherited conditions such as Li-Fraumeni syndrome, Beckwith-Wiedeman syndrome, neurofibromatosis and Costello syndrome. People with any of these syndromes are prone not only to rhabdomyosarcoma but also to other types of cancer and comprise only a minority of patients with rhabdomyosarcoma. Exposure to infections or various environmental factors have also been mentioned as risk factors for rhabdomyosarcoma. However, for the majority of patients, no specific risk factor can be identified. WHAT ARE THE SYMPTOMS OF RHABDOMYOSARCOMA? The signs and symptoms of rhabdomyosarcoma vary depending on what particular area of the body is involved. In the majority of cases a painless lump or mass may be the first sign. Some of these signs and symptoms may include, a droopy eyelid, a bulging eye, or difficulty of the eye to move normally, nasal congestion, bloody nasal discharge, earache or persistent ear discharge, abdominal pain and enlargement, vomiting, constipation, difficulty with urination, a mass protruding out of the vagina, blood in the urine, painless masses in the arms and legs. HOW IS THE DIAGNOSIS OF RHABDOMYOSARCOMA MADE? A careful health history, physical examination with specific attention to the suspected body area involved along with several tests are done to prove the diagnosis of rhabdomyosarcoma, determine the stage and extent of disease. A family history of cancer will be asked, as well as the patients past illnesses and treatments. In most cases, if the patient is seen by a physician soon after the symptoms are noted, the disease is detected early. A piece of tissue is taken (called a biopsy) and is sent for examination under the microscope and use of special tissue stains to identify the cells as rhabdomyosarcoma and to determine its particular type. In some places, the tissue may also be sent for studies to detect genetic abnormalities that are associated with rhabdomyosarcoma. Imaging or radiologic tests are also done to better determine the size and extent of the tumor and to find out if other body areas are involved. These may include X-rays, Computed tomograpy (CT) scans, Magnetic resonance imaging (MRI), Positron emission tomography (PET) scan, bone scan. Other tests include a complete blood count, blood tests to check kidney and liver function, a bone marrow aspirate and biopsy to determine if the disease has spread to the bone marrow. In some cases, a brain scan and spinal fluid examination may be needed, depending to the location of the tumor. HOW DOES ONE STAGE RHABDOMYOSARCOMA? Staging refers to the process used to follow to find out if the rhabdomyosarcoma has spread to other parts of the body. Knowing disease stage is important for treatment planning as well as knowing the child’s chance of cure.