What is rhabdomyosarcoma?
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.
About RhabdomyosarcomaRhabdomyosarcoma 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: -
- 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 mucosal-lined 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.
Types of rhabdomyosarcomaThere are 2 main types of rhabdomyosarcomas.
Embryonal rhabdomyosarcoma : - Embryonal rhabdomyosarcoma (ERMS) is the most common type of rhabdomyosarcoma. It usually affects infants and young children. The cells of ERMS look like the developing muscle cells of a 6- to 8-week-old fetus. ERMS tends to occur in the head and neck area, bladder, vagina, and in or around the prostate and testicles.
Two types of ERMS, botryoid and spindle cell rhabdomyosarcomas, tend to have a better prognosis (outlook) than the more common forms.
Alveolar rhabdomyosarcoma (ARMS) typically affects older children or teenagers and occurs more often in large muscles of the trunk, arms, and legs. ARMS cells look like the normal muscle cells seen in a 10-week-old fetus.
Anaplastic rhabdomyosarcoma and undifferentiated sarcoma
Anaplastic rhabdomyosarcoma (formerly called pleomorphic rhabdomyosarcoma) is a type that occurs in adults but is very rare in children.
Also, some doctors group undifferentiated sarcomas with the rhabdomyosarcomas. Although these uncommon cancers are sarcomas, the cells don't have any features that help classify them further.
What are the symptoms of rhabdomyosarcoma?
The following are the most common symptoms of rhabdomyosarcoma. However, each child may experience symptoms differently. Many symptoms depend on the size and the location of the tumor. Symptoms may not be present until the tumor is very large, especially if it is located deep in the muscle or in the stomach.
Symptoms may include: -
- a tumor or mass that can be seen or felt (may or may not be painful)
- bleeding from the nose, vagina, rectum, or throat (may occur if the location of the tumor is in these areas)
- tingling, numbness, pain, and movement (may be affected if the tumor compresses nerves in the area)
- protrusion of the eye or a drooping eyelid (may indicate a tumor behind the area)
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.
The most 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.
TreatmentRhabdomyosarcoma 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.
For more information, medical assessment and medical quote
as email attachment to
Email : - email@example.com
Contact Center Tel. (+91) 9029304141 (10 am. To 8 pm. IST)
(Only for international patients seeking treatment in India)