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.

There are two staging systems in use for rhabdomyosarcoma. Both are employed in order to determine the

best treatment strategy for the child. The first is called “clinical group”, wherein the disease is based on its

extent and on the completeness of surgical removal. The second is called “staging”, which is in turn based

on the type and size of tumor and on whether nearby lymph nodes or other organs are involved. Both

methods are combined in order to come up with an “overall stage”

HOW IS THE TREATMENT PLANNED AND WHO ARE INVOLVED IN THE TREATMENT?

Once the overall stage is determined, children with rhabdomyosarcoma are then classified into different risk-

based (low, intermediate or high) treatment groups. Risk refers to the chance that a patient will be cured and

whether he or she would need more aggressive treatment.

Patients with low risk disease have an excellent chance of being cured.

For patients with rhabdomyosarcoma, a multidisciplinary team composed of the pediatric oncologist, other

physicians and health professionals trained in the care of children with cancer will be involved. Depending

on what other organs may be affected, physicians such as a pediatric ophthalmologist, pediatric surgeon,

orthopedic oncologist, ear/nose/throat specialist, neurologist, urologist, gynecologist, radiologist, radiation

oncologist, oncology nurses, pharmacists, psychologists, social workers, physical and occupational therapist,

etc. may be involved. Each member of the multidisciplinary care team will contribute to the treatment and

support of the child.

WHAT ARE THE AVAILABLE TREATMENT OPTIONS FOR RHABDOMYOSARCOMA?

The treatment of rhabdomyosarcoma involves the combination of surgery, chemotherapy and radiation

therapy.

Surgery is done for confirmation of the diagnosis and to remove as much tumor as possible while avoiding

loss of function or disfigurement. In cases where removal is not possible, a piece of the tumor is removed for

microscopic examination and confirmation of the diagnosis.

Chemotherapy is the use of drugs to kill cancer cells. It may be given by mouth or injected through a vein or

muscle (systemic therapy). The drugs circulate throughout the body, killing as much cancer cells as possible.

The kind of drug combinations used for the different stages of rhabdomyosarcoma will be discussed by your

physician.

Radiation therapy uses high-energy X-rays or other radiation energy to kill cancer cells. There are several

methods by which radiation therapy may be delivered. They type of method will vary depending on the

patient’s particular need. Radiation therapy is administered by a radiation oncologist.

For patients with high risk disease or those in whom the rhabdomyosarcoma does not respond well to

treatment, studies are now underway on the use of very high doses of chemotherapy followed by

transplantation of blood forming stem cells. For these children, their own stem cells are used and is called an

autologous transplant.

For children with rhabdomyosarcoma who also have genetic risk factors, the management includes genetic

counseling. A geneticist is a specialist trained in discussing with families about inherited diseases.

Counseling is important since some of these patients also have a lifelong risk of developing other cancers.

This information is made possible through the efforts of the Philippine Society of Pediatric

Oncology, Inc. (PSPO), a subspecialty society of the Philippine Pediatric Society (PPS). For

details regarding the treatment of individual patients, it is strongly recommended that they confer

with their pediatric oncologist.