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Together is a new resource for anyone affected by pediatric cancer - patients and their parents, family members, and friends.
Learn MoreSoft tissue sarcoma is a type of cancer that grows in the body’s soft connective tissue including:
Soft tissue sarcomas account for about 7% of all childhood cancers. They can be divided into 2 main types:
Soft tissue sarcomas are named for the tissue that they most resemble:
Rhabdomyosarcoma is more common in younger children. NRSTS is more common in teens. These types of cancers can range from local to highly metastatic (spreading through the body).
Certain conditions passed through families can increase risk for soft tissue sarcomas. Children who have been exposed to radiation are also at higher risk. Treatment for soft tissue sarcoma usually includes surgery, chemotherapy, or radiation therapy.
Rhabdomyosarcoma is a type of soft tissue tumor that often develops in muscle. But it can form anywhere in the body.
It is the most common pediatric soft tissue sarcoma. It usually affects children under age 10. It can even develop before birth.
There are 2 types of rhabdomyosarcoma:
Symptoms of rhabdomyosarcoma depend on the tumor’s location. They can include:
Certain factors may increase risk for rhabdomyosarcoma:
Tests and procedures used to diagnose rhabdomyosarcoma include:
Your child may need other tests, depending on where the tumor is:
Your child might need tests that are not listed here. Your child’s care team will guide this process.
MRI of pediatric rhabdomyosarcoma patient's forearm
MRI of rhabdomyosarcoma patient's abdomen
MRI of pediatric rhabdomyosarcoma patient's abdomen from a cross section, or axial, view
Rhabdomyosarcoma is classified different ways. This can help doctors better understand the disease. It can also help plan the right treatments.
Rhabdomyosarcoma is grouped based on:
| Group | Spread of disease |
|---|---|
| Group I No disease after surgery |
The tumor is limited to one area and is completely removed with surgery. |
| Group II Microscopic disease after surgery |
The tumor is limited to one area and is removed with surgery, but cancer cells are found in nearby tissue that surrounded the tumor (margin) and/or lymph nodes. |
| Group III Significant disease after surgery |
The tumor is limited to one area but cannot be completely removed with surgery. |
| Group IV Metastatic disease |
The tumor has spread to other parts of the body at diagnosis (metastatic). |
Rhabdomyosarcoma can also be staged using the TNM system. This system considers 3 factors:
Rhabdomyosarcoma can be divided into 4 stages using the TNM system.
| Stage | Location | Tumor Size | Lymph Nodes | Metastasis |
|---|---|---|---|---|
| Where is the primary tumor? | How large is the tumor? |
Are cancer cells found in nearby lymph nodes? | Has the cancer spread to other parts of the body? | |
| 1 | Favorable: Tumor is in the area of the eye, head or neck or in the genitorurinary tract Exceptions: Tumor is located near the brain or spinal cord or in the bladder or prostate |
Any size |
Yes or No | No |
| 2 | Unfavorable: All other sites |
Less than 5 cm |
No | No |
| 3 | Unfavorable: All other sites | Less than 5 cm Greater than 5 cm |
Yes Yes or No |
No |
| 4 | Any site |
Any size | Yes or No | Yes |
Rhabdomyosarcoma requires a combination of treatments. These can include:
All children will have chemotherapy as part of rhabdomyosarcoma treatment. This helps treat microscopic spread of the cancer.
Tumors that can be seen on imaging tests may be treated with a combination of surgery and radiation therapy.
The chance of recovery from rhabdomyosarcoma depends on:
Doctors classify rhabdomyosarcoma patients into risk groups to help plan treatments.
Risk classification helps doctors decide:
If rhabdomyosarcoma comes back, the prognosis may be affected by:
Children with rhabdomyosarcoma that has not spread have greater than 70% chance of long-term cure. About 20% of patients have disease that has already spread at diagnosis.
If the cancer has already spread at diagnosis, the chance of survival is less than 30%.
About 30% of patients with rhabdomyosarcoma will have disease that returns after treatment. The disease may return at the location of the initial tumor or in other sites.
Recurrent rhabdomyosarcoma is difficult to treat. The chance of cure is low. Clinical trials may be an option.
Balancing quality of life with cancer-directed therapy is important. Talk to your care team about what problems to expect and ways to help manage them.
"Two years off therapy, we are cancer free but still not at the 5-year mark. As time passes, I find that my worries change. The stress of cancer is still there. 'Scanxiety' hits as our 6-month checkup time gets closer. But what I look for now are more post-therapy effects, especially related to proton beam radiation because it just hasn’t been around long enough to know what to expect."
– Michelle, mom to Reid
Reid was treated for two rare cancers behind his eye: rhabdomyosarcoma and ectomesenchymoma. His treatments included surgery, chemotherapy, and proton beam radiation.
Soft tissue sarcomas can return years later. The tumor can come back in the same place or in another part of the body.
Your child will need follow-up care to screen for recurrence after treatment ends. The medical team will tell you which tests are needed and how often your child should have checkups.
Children treated for soft tissue sarcoma are at risk for late effects related to therapy. Specific problems depend on the location of the tumor and the type of therapy your child received.
Long-term effects of surgery can include loss of function and changes in appearance.
Survivors who were treated with radiation therapy may have growth problems, bone disease, and other issues due to treatment. They may also have a higher risk of second cancers.
Survivors who were treated with systemic chemotherapy should be monitored for:
All survivors should continue to have regular physical checkups and screenings by a primary physician. A healthy lifestyle and eating habits can also help.
—
Reviewed: December 2022
