What Is It?
Sarcomas are a type of cancer that develops from the fleshy parts of the body. Cancers from the soft tissues are sarcomas. (This type of cancer is different from a carcinoma, which develops in glandular tissue, for example, lung cancer, colon cancer pancreatic cancer, breast cancer, prostate cancer.) Rhabdomyosarcoma is a cancer that forms in the skeletal muscles. Skeletal muscles attach to bones. They help the body move. Most rhabdomyosarcomas occur in children and teenagers.
Rhabdomyosarcoma can occur anywhere in the body. It typically appears in the arms, legs, head and neck, bladder, reproductive organs, chest, and abdomen. Rhabdomyosarcoma can spread to other organs.
There are two main types of rhabdomyosarcoma:
Embryonal rhabdomyosarcoma usually affects children under age 6.
Alveolarrhabdomyosarcoma usually affects older children or teenagers. It tends to be more aggressive than embryonal rhabdomyosarcoma.
Symptoms
The signs of rhabdomyosarcoma depend largely on the size and location of the cancer. These tumors may not cause symptoms until they are large.
Common symptoms include:
Persistent lump or swellingin the body that may be painful
Bulging of the eye or a drooping eyelid
Headache and nausea
Trouble urinating or having bowel movements
Blood in the urine
Earache or sinus infection symptoms
Bleeding from the nose, throat, vagina, or rectum
Vomiting, abdominal pain, constipation
These symptoms do not necessarily mean cancer. But it is important to contact your doctor if you or your child experience any of them.
Diagnosis
Your doctor will look for signs of disease with a physical exam and medical history. He or she will ask about your health habits and past illnesses and treatments.
Your doctor may also recommend one or more of the following tests: Diagnosis will also require some combination of the following tests and procedures:
X-ray. Uses energy beams to make pictures of tissues, bones, and organs.
Computed tomography (CT) scan. Uses a rotating x-ray camera to take detailed, cross-sectional pictures of the tumor as well as to see if the tumor has spread to other organs (for example, the lungs or abdomen).
Magnetic resonance imaging (MRI). Employs a strong magnet and radio waves to take detailed pictures inside the body.
PET scan. Doctors sometimes use this test to see if and where the cancer may have spread.
Bone scan. Uses a scanner and low-level radioactive material to detect whether cancer cells have spread to the bone.
Biopsy. Surgery in which cells or tissues are removed for examination. Biopsies can be performed using a needle or by cutting through the skin. Specially trained doctors called pathologists can then study the tissue under the microscope.
Bone marrow aspiration and biopsy. Bone and bone marrow fluid is removed from the bones of the lower back and pelvis to see if the cancer has spread.
Staging
Staging assesses how much cancer exists, and where and how far it has spread. The stage of rhabdomyosarcoma is based on three factors:
Type of rhabdomyosarcoma
Embryonal or alveolar
Clinical group
Rhabdomyosarcoma is assigned a clinical group (I-IV). The group is based on the extent of the disease and how much tumor is initially removed during surgery.
TNM rating
The TNM rating is based on:
T (tumor size)
N (whether the cancer has spread to nearby lymph nodes)
M (whether the cancer has spread to distant organs)
Doctors will then classify the patient as low, intermediate, or high risk.
Expected Duration
Rhabdomyosarcoma will continue to grow until treated. If left untreated, it may spread elsewhere in the body.
Prevention
Certain inherited conditions increase a person's risk of developing rhabdomyosarcoma. These include range of genetic abnormalities that make people more likely to develop this type of cancer as well as cancers:
Li-Fraumeni syndrome
Neurofibromatosis type 1
Beckwith-Wiedemann syndrome
Costello syndrome
Noonan syndrome
There are no known preventable risk factors for this disease. Genetic testing may help identify other family members who may be at risk for this type of sarcoma or other types of cancers.
Treatment
Your doctor will recommend treatment based on several factors, including:
Location and extent of the tumor
Patient's age
Patient's ability to tolerate the therapies, many of which can have serious side effects
Treatment usually involves some combination of surgery, chemotherapy, and radiation therapy.
The surgeon removes as much of the tumor as possible. He or she will try to minimize damage or disfigurement when doing so, but that can be difficult. In some case, the surgeon must remove all or part of a limb. In other cases, the limb can be saved.
Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy is almost always given after surgery. It destroys any remaining cancer cells where the tumor was removed. It can also kill small pockets of cancer cells that may be present in other parts of the body.
The doctor may recommend radiation therapy for some patients. Radiation therapy uses high-energy radiation to kill cancer cells and shrink tumors. It may be recommended if the entire tumor was not removed during surgery. It may also be used if the cancer is higher risk but has not spread to distant sites.
Treatments for rhabdomyosarcoma may cause side effects. These include hair loss, nausea, fatigue, and increased susceptibility to infection. Your doctor will help you to manage these side effects. Longer term side effects can also occur later on in life. This can include the early development of heart disease or problems with memory. Again all of these side effects are generally well tolerated. Doctor's will choose the treatment most likely to help cure the patient of the sarcoma, and without treatment, the disease may be fatal.
Treating cancer in children requires special considerations. The medical team should consider and try to limit the possible long-term side effects of cancer treatment. These include:
Problems with fertility
Increased risk of developing a second cancer
Problems with bone growth
Problems with the growth of soft tissues
Damage to the heart
Problems with mental ability
When to Call a Professional
Call your doctor if you or your child experience any symptoms of rhabdomyosarcoma including:
A persistent lump or swellingin part of the body
Bulging of the eye or a swollen eyelid
Headache and nausea
Trouble urinating or having bowel movements
Blood in the urine
Bleeding from the nose, throat, vagina, or rectum
Enlargement of one side of the scrotum
Prognosis
The outlook depends on many factors, including:
The tumor's type, location, and size
Whether the tumor can be surgically removed
Whether the cancer has spread
Patient's age and general health
The particular characteristics of the tumor cells
It is important to get prompt medical attention and continuous follow-up care for rhabdomyosarcoma. Because these cancers are relatively rare, it is very important to get treatment at a hospital with experience treating children with this type of cancer. Pediatric oncologists are constantly striving to improve the outcomes of young patients with this disease. Your doctor may even suggest that your child participate in a study testing new therapies for this type of sarcoma.
Additional Info
National Cancer Institute (NCI)
NCI Public Inquiries Office
6116 Executive Blvd.
Room 3036A
Bethesda, MD 20892-8322
Toll-Free: 1-800-422-6237
TTY: 1-800-332-8615
http://www.nci.nih.gov/
American Cancer Society (ACS)
Toll-Free: 1-800-227-2345
TTY: 1-866-228-4327
http://www.cancer.org/
Sarcoma Foundation of America
9884 Main Street
P.O. Box 458
Damascus, Maryland 20872
Phone: 301-253-8687
Fax: 301-253-8690
www.curesarcoma.org
Children's Hospital Boston
300 Longwood Ave.
Boston, MA 02115
Phone: 617-355-6000
TTY: 617-355-0443
www.childrenshospital.org
Dana-Farber Cancer Institute
44 Binney St.
Boston, MA 02115
Toll-Free: 1-866-408-3324
TY: 617-632-5330
http://www.dana-farber.org
