What is Rhabdomyosarcoma?
Rhabdomyosarcoma is a cancer, or sarcoma, whose cells have features of muscle cells. Rhabdomyosarcoma can develop anywhere in the body. The tumor can occur at any age but is most common in children between the ages of 1 and 5 years.
Research is being done to try and find out the cause of and best type of treatment for this cancer.
Signs and Symptoms of Rhabdomyosarcoma
Symptoms depend on where the tumor develops. The common sites are:
The Head and the Neck
In this area the tumor may arise inside the nose or sinuses, and middle ear. This may cause nasal / sinus obstruction, congestion, nasal discharge, a noticeable mass protruding from the ear, or discharge from the nose or it may appear as a mass protruding from the ear.
Eye Socket or Orbit
In this site, the tumor will cause the eye to protrude or to appear larger and/ or the eye muscles may not work normally.
Muscles of the Arms or Legs
The tumor will show up as a lump or mass in these areas.
Trunk of the Body
If the tumor occurs on the surface muscle of the chest or abdomen it will present as a visible mass. If it begins in the muscles inside the body it will present as a mass which may or may not be felt. It may be discovered accidentally by you while bathing the child or by the doctor during a routine examination. Sometimes the mass may be found when the tumor presses on some other structure and causes a problem. For example, tumor may compress the rectum leading to constipation.
Genitourinary System
The tumor can arise in the bladder muscle causing problems with passing urine. It can also begin in the vagina and present as a mass like a bunch of grapes at the entrance to the vagina. Bleeding from the vagina or a discharge may also be present. It can also arise in the testes or prostate in males.
Rhabdomyosarcoma Diagnosis
The only way the physician can determine your child definitely has rhabdomyosarcoma is to examine a piece of the tumor after surgery. The type of operation your child will have depends on the site of the tumor. If the tumor is in an inaccessible site, such as the eye or ear, the surgeon will just take a very small piece for diagnosis to avoid damaging the surrounding structures.
Your child may have X-rays and scans before surgery to help the surgeon see the extent of the tumor. The piece of tissue obtained at surgery is examined carefully under the microscope. Sometimes it is difficult to diagnose exactly which type of cancer is present, because rhabdomyosarcoma can spread in three ways:
- By direct extension. The tumor stays in its original site and location and grows until it comes into contact with another organ or tissue which it invades.
- Via the lymphatic system to the lymph nodes.
- By invading the bloodstream and spreading to any area of the body. Rhabdomyosarcoma tends to spread or metastasize to the lungs, liver, bone, and bone marrow. Tumors involving the ear, nose, or sinus have a tendency to spread into the central nervous system.
Staging is determined by how far the tumor has spread in your child's body. The doctor determines the staging of your child's tumor by looking carefully to see if other organs or lymph nodes are involved. X-rays, CT scans, PET scans, bone scans and MRIs are used to check the areas of the body to which rhabdomyosarcoma tends to spread, such as the liver, lungs and bones.
Bone marrow aspirations and biopsies are done to assess spread to the bone marrow. A spinal tap may be indicated if the tumor is in the ear, sinuses, or nose. CT or MRI of the brain will also be taken, because tumor in these sites has a tendency to spread to the brain.
Long-term Outlook
Prognosis depends on the stage of the disease. More than 70 percent of children with localized rhabdomyosarcoma can be cured of their disease. Children with metastases to other organs (Stage IV disease) have a significantly reduced chance of long-term survival, even if the cancer goes into remission with initial therapy.
Oncologists are cautious about using the word "cure." It has been shown that if a child with rhabdomyosarcoma is living two years after diagnosis with no evidence of tumor, the chance of the cancer coming back is small. However, these children will have to be watched carefully for many years to see if they are really cured.
Rhabdomyosarcoma Treatment
Treatment for rhabdomyosarcoma may involve surgery, radiation therapy and chemotherapy − alone or in combination.
In some situations, the doctor may recommend proton therapy instead of traditional radiation therapy. Proton therapy targets the tumor while avoiding organs and healthy tissue. This means fewer short-term side effects and long-term complications from radiation. Cincinnati Children’s is one of only a few pediatric hospitals in the country that offers proton therapy.
Your child may receive treatment as an inpatient and / or as an outpatient, depending on the stage of the disease and the phase of therapy. We will do periodic blood tests to check white blood cell, red blood cell and platelet counts, and test for any signs of returning tumor. Every few months, we will take X-rays of the lungs, liver and bones.
