Rhabdomyosarcoma is a malignant (cancerous) soft tissue tumor found most often in children. The most common sites are the structures of the head and neck, the urogenital tract, and the arms or legs.
Rhabdomyosarcoma is a cancerous tumor that originates in the soft tissues of the body, including the muscles, tendons, and connective tissues. The most common sites for this tumor to be found include the head, neck, bladder, vagina, arms, legs, and trunk. Rhabdomyosarcoma can also be found in places where skeletal muscles are absent or very small, such as in the prostate, middle ear, and bile duct system. The cancer cells associated with this disease can spread (metastasize) to other areas of the body.
Embryonal rhabdomyosarcoma, the most common type, usually occurs in children under 6 years of age. Alveolar rhabdomyosarcoma occurs in older children and accounts for about 20 percent of all cases.
Rhabdomyosarcoma accounts for about 3 percent of childhood cancers. In the US, about 350 children are diagnosed with rhabdomyosarcoma each year. This disease affects children between the ages of 2 to 20 years of age, but can occur at any age. For unknown reasons, males are affected slightly more often than females.
The body contains 3 main types of muscles
- Skeletal (voluntary) muscles are muscles that we control to move parts of our body.
- Cardiac muscle is the main muscle type in the heart.
- Smooth muscle is present in internal organs (except for the heart). For example, smooth muscles in the intestines push food along as it is digested. We do not control this movement.
What causes RMS?
In most cases, there are no clear predisposing risk factors for the development of RMS. However, it has been associated with other conditions, including :
- Neurofibromatosis type 1
- Beckwith-Wiedemann syndrome
- Li-Fraumeni syndrome
- Cardio-facio-cutaneous syndrome
- Costello syndrome
What are the symptoms of rhabdomyosarcoma?
Most often rhabdomyosarcoma presents as a mass, however the signs and symptoms depend on the location of the primary tumor:
- Genitourinary tumors may result in blood in the urine, difficulty urinating or bleeding from the vagina. They can also cause a mass next to the testicle in the scrotum, or in the vagina.
- Parameningeal tumors (tumors adjacent to the base of the skull) may cause facial asymmetry, difficulty breathing through or drainage from one nostril, ear discharge, headaches or facial pain. Swelling around the eye is common with tumors of the orbit.
- Extremity (arm and leg) tumors often present as a rapidly enlarging, firm lesion.
It is now possible to cure nearly 3 of every 4 children with rhabdomyosarcoma. Prognostic factors are characteristics of the patients and their tumors that are designated as more or less “favorable” based on the response to treatment and outcome of hundreds of patients treated over decades. These factors also are used to determine the best choice of therapy. Although the “unfavorable” forms require more intensive treatment than those considered “favorable,” all RMS are treated with chemotherapy and surgery; sometimes radiation therapy is added.
The most important prognostic factors include:
- Extent of the disease: This is defined by the Intergroup Rhabdomyosarcoma Study (IRS) Stage and Group systems.
- Primary tumor site: Some tumor sites are defined as favorable. These include tumors that begin in the orbit, the head and neck and the bladder. All other sites including parameningeal tumors and non-bladder genitourinary sites are classified as unfavorable.
- Histology subtype: Embryonal histology tumors have a more favorable outcome.
- Age: Patients under one and over 10 years of age have a somewhat less favorable prognosis.
- Presence of distant metastasis: Patients whose tumors have metastasized (spread to other areas) at the time of diagnosis have a less favorable prognosis.
How is rhabdomyosarcoma diagnosed?
In addition to a complete medical history and physical examination, diagnostic procedures for rhabdomyosarcoma may include:
- biopsy of the tumor - when a sample of tissue is removed from the tumor and examined under a microscope.
- blood and urine tests
- multiple imaging studies:
- computed tomography scan (Also called a CT or CAT scan.) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
- magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body.
- x-ray - a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
- ultrasound (Also called sonography.) - a diagnostic imaging technique that uses high-frequency sound waves and a computer to create images of blood vessels, tissues, and organs. Ultrasounds are used to view internal organs as they function, and to assess blood flow through various vessels.
- bone scans - pictures or x-rays taken of the bone after a dye has been injected that is absorbed by bone tissue. These are used to detect tumors and bone abnormalities.
- bone marrow aspiration and/or biopsy - a procedure that involves taking a small amount of bone marrow fluid (aspiration) and/or solid bone marrow tissue (called a core biopsy), usually from the hip bones, to be examined for the number, size, and maturity of blood cells and/or abnormal cells.
- lumbar puncture/spinal tap - a special needle is placed into the lower back, into the spinal canal. This is the area around the spinal cord. The pressure in the spinal canal and brain can then be measured. A small amount of cerebral spinal fluid (CSF) can be removed and sent for testing to determine if there is an infection or other problems. CSF is the fluid that bathes your child's brain and spinal cord.
Treatment for rhabdomyosarcoma
Specific treatment for rhabdomyosarcoma will be determined by your child's physician based on:
- your child's age, overall health, and medical history
- extent of the disease
- your child's tolerance for specific medications, procedures, or therapies
- expectations for the course of the disease
- your opinion or preference
Treatment may include (alone or in combination):
- resection of tumor and/or metastases
- radiation therapy
- lumbar puncture (to administer medication and treat cancer cells if found in this site)
- supportive care (for the side effects of treatment)
- antibiotics (to prevent/treat infections)
- bone marrow transplant or stem cell transplantation
- continuous follow-up care (to manage disease and detect any recurrence of the tumor)
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