Rhabdomyosarcoma is a cancerous tumor that grows in the soft tissues of the body, particularly in the muscles that attach to bone and help the body to move.
- The most common sites for this tumor to be found include the head, neck, bladder, vagina, arms, leg, 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 to other areas of the body.
In good hands
Children and teenagers with rhabdomyosarcoma are treated through the Bone and Soft Tissue Tumor Program at Dana-Farber/Boston Children's Cancer and Blood Disorders Center. Dana-Farber/Boston Children's specialists are known for treating children with the most complex cases as well as for their expertise in delivering specialized treatments. We take an in-depth, multidisciplinary approach to care that ensures that your child receives a personalized treatment plan—as well as supportive care before and after his treatment. For your child’s care, we draw on the expertise of specialists such as:
- pediatric orthopedic and general surgeons who are nationally recognized for their expertise in removing bone and soft tissue tumors
- pediatric experts from every needed medical subspecialty, including pathology, radiology, physical therapy and bracing/casting
- experienced pediatric nurses
- Child Life specialists, psychologists, social workers and resource specialists
How common is rhabdomyosarcoma?
- Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood. In the United States, about 250 children are diagnosed with rhabdomyosarcoma each year.
- For unknown reasons, males are affected slightly more often than females.
- Embryonal rhabdomyosarcoma, the most common type of rhabdomyosarcoma, usually occurs in children under 6 years old.
- Alveolar rhabdomyosarcoma occurs in older children and accounts for about 20 percent of all cases.
What causes rhabdomyosarcoma?
Most doctors believe that some rhabdomyosarcoma tumors begin developing in the fetus. Rhabdomyoblasts are the cells at the initial stages of development of an unborn baby. These cells will mature and develop into muscles.
Rhabdomyosarcomas usually have some type of chromosome abnormality in the cells of the tumor. There has been no association between rhabdomyosarcoma and environmental exposures.
What kind of symptoms will my child have?
Each child experiences 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.
Your child's 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?
Your child's doctor will do a complete medical and physical examination. In addition, some or all of these tests will be prescribed:
- blood and urine tests - including a complete blood count
- multiple imaging studies - to evaluate the primary tumor and determine the extent/location of any spreading, including:
- Computerized tomography scan (also called a CT or CAT scan) - a CT scan shows detailed images of any part of the body, including the bones, muscles and fat.
- Magnetic resonance imaging (MRI) - a test 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.
- Ultrasound (also called sonography) - 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 biopsy and/or aspiration - a procedure that involves taking a small amount of bone marrow fluid and tissue, usually from part of the hip bones, to examine the number, size, and maturity of blood cells and/or abnormal cells.
- spinal tap/lumbar puncture - a procedure that involves inserting a special needle through the lower back into the spinal canal, 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.
- biopsy - a sample taken of the primary tumor and/or metastatic lesions.
Staging the tumor
Diagnosing rhabdomyosarcoma also involves staging and classifying the disease, which will help your child's doctor determine treatment options and prognosis. Staging is the process of determining whether cancer has spread and, if so, how far.
There are various staging systems that can be used for rhabdomyosarcoma. Talk to your child's physician for information on staging.
Treatment and care
How is rhabdomyosarcoma treated?
Treatment for rhabdomyosarcoma requires close coordination between surgeons, pediatric oncologists and radiotherapists. The response of tumors is very much dependent on their site of origin.
A series of studies have been performed by the Intergroup Rhabdomyosarcoma Study (IRS), which has outlined the treatment of rhabdomyosarcoma. Boston Children's plays an active role in this organization.
We'll generally do surgery to both provide a diagnosis of the tumor type as well as give your child's doctor information regarding the stage of the tumor. Treatment with chemotherapy, with or without radiation therapy, is then determined by the extent of removal of the tumor as well as by its stage and location.
- A tumor in the bladder or prostate requires chemotherapy prior to attempts at surgical removal or treatment with radiation.
- A tumor in the muscles of the arms or legs is often treated initially with surgical removal, which may be followed by chemotherapy, with or without radiation.
- Tumors around the eye are very responsive to chemotherapy and radiation, so they rarely require surgical removal.
In addition to surgery, treatment for malignant odontogenic tumors may include:
- Radiation therapy - This treatment can help stop the growth of abnormal cells in specific areas of the body. Radiation therapy uses high-energy rays (radiation) from a specialized machine to damage or kill abnormal cells.
- Chemotherapy - This treatment is used in cases of advanced or aggressive tumor growth. This treatment can help stop the growth of abnormal cells throughout the body. Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill or slow the growth of targeted cells.
- Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
- Often, a combination of chemotherapy drugs is used.
- Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.
Chemotherapy can be given:
While chemotherapy can be quite effective in treating certain cancers, the agents don't completely differentiate normal healthy cells from abnormal cells. Because of this, your child could have adverse side effects during treatment.
- as a pill to swallow
- as an injection into the muscle or fat tissue
- intravenously (directly to the bloodstream)
- intrathecally (directly into the spinal column with a needle)
- Lumbar puncture - To administer medication and treat cancer cells directly if found in this site.
- Stem cell transplant - A treatment involving stem cells, a specific type of cell from which all blood cells develop. Transplantation of normal stem cells from another person is used to help restore normal blood production in patients whose own ability to make any or all of these blood cells has been compromised by cancer, intensive cancer treatment, or other types of damage or abnormality.
- Stem cell transplantation and the treatment needed to manage its effects are very complex. Your physician will give you more detailed information on what to expect.
What's my child's long-term outlook?
It varies widely from child to child and greatly depends on:
- the extent of the disease
- the size and location of the tumor
- the tumor's characteristics when examined under a microscope
- the presence or absence of metastasis
- the tumor's response to therapy
- the age and overall health of your child
- your child's tolerance of specific medications, procedures, or therapies
- new developments in treatment
Prompt medical attention and aggressive therapy are important for the best prognosis.
What is the recommended long-term care for children treated for rhabdomyosarcoma?
Your child should visit a cancer survivorship program every year to:
- manage disease complications
- screen for early recurrence of cancer
- manage late effects of treatment
A typical follow-up visit may include some or all of the following:
- a physical exam
- laboratory testing
- imaging scans
Through the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber/Boston Children's, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.
- Our childhood cancer survivorship clinic is held weekly.
- In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.
- We also offer the following services:
- patient and family education
- psychosocial assessment
- genetic counseling
- reproductive and fertility evaluation and counseling
- opportunities to speak with other childhood cancer survivors