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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 muscle, nerves, tendons, fat, and walls of blood and lymph vessels. Soft tissue sarcomas can be divided into two main types:
Soft tissue sarcomas account for about 7% of all childhood cancers. Rhabdomyosarcoma is more common in younger children, and NRSTS is more common in adolescents. The clinical behavior of soft tissue sarcomas can range from locally aggressive to highly metastatic.
Soft tissue sarcomas are named for the tissue which they most resemble:
Certain inherited conditions can increase risk for soft tissue sarcomas. Children who have been exposed to ionizing radiation are also at higher risk. Treatment for soft tissue sarcoma usually includes surgery, chemotherapy, and/or radiation therapy.
Rhabdomyosarcoma is a type of soft tissue tumor that often develops in muscle. However, it can form anywhere in the body and is the most common pediatric soft tissue sarcoma. It usually affects children under 10 years of age and can develop before birth.
There are two types of rhabdomyosarcoma:
Certain factors may increase risk for rhabdomyosarcoma including age, gender, and ethnicity.
Symptoms of rhabdomyosarcoma depend on where the tumor is located. Signs and symptoms of rhabdomyosarcoma include:
Several types of procedures and tests are used to diagnose rhabdomyosarcoma. These include:
Depending on the location of the tumor, additional tests may be needed including:
Site-specific evaluations may be needed depending on tumor location. A multidisciplinary medical team is important to provide optimal care throughout treatment.
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
In rhabdomyosarcoma, there are different ways the disease can be classified. This can help doctors better understand the disease and plan appropriate treatments.
Rhabdomyosarcoma is grouped based on tumor location, spread of disease, and result of surgery.
| 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:
Using the TNM System, rhabdomyosarcoma can be divided into 4 stages.
| 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 |
The chance of recovery from rhabdomyosarcoma depends on several factors:
Doctors classify rhabdomyosarcoma patients into risk groups to help plan treatments. Groups are based on the stage of disease and the ability of surgery to remove the tumor.
This information helps doctors decide what treatments to use, dose of medication, and the number of treatments needed.
If rhabdomyosarcoma has recurred, prognosis may be influenced by where in the body the cancer came back, how much time passed between the original cancer and recurrence, and what therapies were originally used to treat the cancer.
Children with localized rhabdomyosarcoma have greater than 70% chance of long term cure. About 20% of patients have metastatic disease at diagnosis. If the cancer has already spread at diagnosis (metastatic rhabdomyosarcoma), the chance of survival is less than 30%.
Approximately 30% of patients with rhabdomyosarcoma will have recurrence of cancer. Rhabdomyosarcoma may recur at the location of the initial tumor or in other sites. Recurrent rhabdomyosarcoma is difficult to treat, and the chance of cure is low.
Rhabdomyosarcoma requires a combination of treatments including chemotherapy, surgery, and/or radiation therapy. All children receive systemic chemotherapy as part of treatment for rhabdomyosarcoma. This is important to treat microscopic spread of disease that may not be detected. Tumors visible on imaging are also treated with surgery and/or radiation therapy.
The chemotherapy regimen for rhabdomyosarcoma includes vincristine, actinomycin D, and cyclophosphamide and irinotecan. A standard course of treatment is 14-16 cycles (period of treatment followed by period of rest).
Other treatments include other chemotherapy drugs like doxorubicin and experimental agents such as targeted therapies, drugs that inhibit the cell cycle, and immunotherapy.
Many children are offered treatment for rhabdomyosarcoma as part of a clinical trial.
"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 recur years into survivorship. The tumor can come back in the same place (local recurrence) or in another part of the body (distant recurrence).
Patients will receive follow-up care to screen for recurrence after treatment ends. The medical team will make specific recommendations for the types of tests needed and how often patients should be seen for 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 received.
Long-term effects of surgery can include loss of function and changes in appearance.
Survivors treated with radiation therapy may have impaired growth, bone disease, and other problems due to treatment as well as increased risk of second cancers.
Survivors treated with systemic chemotherapy should be monitored for medication-specific late-effects which may include heart problems, kidney damage, infertility, endocrine dysfunction, and risk of second cancers.
All survivors should continue to have regular physical checkups and screenings by a primary physician. For general health and disease prevention, survivors should adopt healthy lifestyle and eating habits.
—
Reviewed: June 2018
