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Rhabdomyosarcoma
What is Rhabdomyosarcoma?
Rhabdomyosarcoma is a cancer of striated muscle tissue. Rhabdomyosarcoma can develop
wherever there is this type of muscle tissue. The tumour can occur at any age, but is most
common in children between the ages of one and five years.
What causes Rhabdomyosarcoma?
No one knows the answer to this question. Research investigators are trying to find out
what causes this cancer. Remember that you are not to blame, and that the tumour is not
contagious.
What are the symptoms of Rhabdomyosarcoma?
Since striated muscle tissue is present all over the body, rhabdomyosarcoma can occur
anywhere in the body. Symptoms depend on where the tumour develops. The most common sites
are:
- The Head and Neck:
In this area, the tumour may arise inside the nose or sinuses and cause a blocked nose or discharge from the nose or it may appear as a mass protruding from the ear. - The Eye Socket or Orbit:
In this site, the tumour will cause the eye to protrude. - The Muscles of the Arms or Legs: The tumour will show up as a lump in these areas.
- Trunk of the Body:
If the tumour occurs in the surface muscle of the chest or abdomen it will present as a visible lump. If it arises in the muscles inside the body it will present as a lump which can only be felt. Often this lump is felt accidentally by you while bathing your child or by your doctor during a routine examination. Sometimes the lump may press on some other structure causing a problem. It may press on the bladder and cause trouble in passing urine. It may compress the rectum leading to constipation. - Genitourinary System (organs and parts
concerned with the kidneys):
The tumour can arise in the muscle of the bladder giving problems with passing urine. It can start out in the vagina and present like a mass like a bunch of grapes at the entrance to the vagina. It can arise in the testes and prostate in males.
The tumour may spread to other parts of the body, especially the lungs, brain, bones and bone marrow. The first sign that your child has rhabdomyosarcoma may be symptoms of disease in one of these sites.
How is Rhabdomyosarcoma Diagnosed?
The only way to tell that your child definitely has rhabdomyosarcoma is to examine a piece
of the tumour after surgery. The type of operation your child will have depends on the
site of the tumour. If the tumour is in an accessible site such as the abdomen, the
surgeon will remove as much tumour as possible. If the tumour is in an inaccessible site
such as the eye, the surgeon will just take a very small piece for diagnosis (biopsy)
because they would not want to cause damage to other structures. The piece of tissue
obtained at surgery is examined carefully under the microscope. Sometimes it is difficult
to diagnose exactly which type of cancer your child has. Your child will have x-rays and
scans of the site where the lump arises and also a CT scan of the chest and a bone marrow
biopsy before surgery to help determine whether the tumour has spread.
How is Rhabdomyosarcoma Staged?
Staging is determining how far the tumour has spread in your child's body. Staging is
important as it determines what type of treatment your child will have and help the doctor
give you an idea of how well your child will do.
Rhabdomyosarcoma can spread in three ways:
- It can spread by direct extension. The tumour stays in its original site and grows until it comes into contact with another organ or tissue which it invades.
- The tumour can spread via the lymphatic system to the neighbouring lymph nodes.
- It can invade the bloodstream and spread to other areas of the body. Rhabdomyosarcoma tends to metastasize to the lung, bone, brain and bone marrow. Tumour involving the ear, nose or sinuses has a special tendency to spread to the central nervous system.
Staging
The doctor will determine the stage of your child's tumour by:
- Looking carefully to see if other organs or lymph nodes are involved.
- X-raying and scanning the areas of the body that rhabdomyosarcoma tends to spread to.
- Bone marrow examination.
- If the tumour is in the ear, sinuses or nose, a spinal tap will be done. X-rays of the brain will also be taken. This is because tumour in these sites has a special tendency to spread to the brain.
- Lung CT Scan.
- Gallium and bone scans (undertake in the nuclear medicine department).
Based on the information obtained from the above procedures your child will be placed in one of the following stages:
Stage I: The tumour has been completely removed at surgery. No lymph node involvement.
Stage II: Microscopic tumour has been left behind at surgery or lymph nodes are involved.
Stage III: Gross tumour (tumour you can see with your eyes) has been left behind at surgery.
Stage IV: The tumour has spread to other areas of the body.
What is the prognosis for a child with Rhabdomyosarcoma?
The most important prognostic factor is the stage of the disease.
- Children with Stage I or II disease have a very good chance of long-term survival.
- Children with Stage III disease have a good chance of long-term survival.
- Children with Stage IV disease have a chance of long-term survival, but not as good as that of children with Stage I-III disease.
Oncologists are cautious about using the word "cure". It has been shown that if a child with rhabdomyosarcoma is alive three and a half years from diagnosis with no evidence of tumour, the chance of the cancer ever returning is very small. However, these children will have to be watched carefully for many years to see if they really are cured.
Other factors influencing prognosis are:
If your child has the primary tumour in the nose, ear, or sinuses, he/she will have a
spinal tap and x-rays of the head to see if the central nervous system has been invaded by
the tumour. If it has, he/she will receive treatment to the central nervous system. This
treatment consists of:
- Radiation treatment of the brain.
- Spinal taps with medicines given into the spinal canal. These medicines will be methotrexate, hydrocortisone, and cytosine arbinoside.
The amount of treatment will depend on the amount of tumour in the central nervous system. Your doctor will discuss this with you.
How will my child be followed medically?
Most, if not all, of your child's treatment will be given as an out-patient. He/she will
come to the radiation therapy department for the radiation treatment and to the oncology
clinic for chemotherapy. A blood count will be done before each course of drugs is given
to make sure that your child's white blood cell, red blood cell, and platelet counts are
normal.
Your child will be examined for side effects of treatment and for any sign of the tumour returning. Every few months your child will have x-rays and scans of the lungs, liver and bones.
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