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Soft Tissue Sarcoma

What is soft tissue sarcoma?

Soft tissue sarcoma is a type of cancer that grows in the body’s soft connective tissue including:

  • Muscle
  • Nerves
  • Tendons
  • Fat
  • Walls of blood and lymph vessels

Soft tissue sarcomas account for about 7% of all childhood cancers. They can be divided into 2 main types:

  • Rhabdomyosarcoma
  • Non-rhabdomyosarcoma soft tissue sarcoma (NRSTS)

Soft tissue sarcomas are named for the tissue that they most resemble:

  • Skeletal muscle: rhabdomyosarcoma
  • Smooth muscle: leiomyosarcoma
  • Cartilage: chondrosarcoma
  • Fat: liposarcoma
  • Blood and lymph vessels: angiosarcoma
  • Peripheral nerves: malignant peripheral nerve sheath tumor
  • Fibrous tissue: fibrosarcoma

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.

 

  1. Rhabdomyosarcoma

    What is rhabdomyosarcoma?

    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:

    • Embryonal rhabdomyosarcoma – This tumor is most common in children under age 5 and often forms in the head, neck, bladder, vagina, prostate, or testicles.
    • Alveolar rhabdomyosarcoma – This tumor affects all age groups and usually forms in the larger muscles of the arms, legs, and trunk of the body.

    Symptoms of rhabdomyosarcoma

    Symptoms of rhabdomyosarcoma depend on the tumor’s location. They can include:

    • Swelling or a lump that does not go away, sometimes painful
    • Headaches
    • Bulging of the eye
    • Bleeding in the nose, vagina, or rectum
    • Constipation or difficulty urinating

    Risk factors of rhabdomyosarcoma

    Certain factors may increase risk for rhabdomyosarcoma:

    • About 2/3 of rhabdomyosarcoma tumors happen in children under age 10.
    • Rhabdomyosarcoma occurs slightly more often in males than females.
    • White children have a higher risk of rhabdomyosarcoma than Black children.
    • Certain inherited conditions can increase risk for rhabdomyosarcoma:

    Diagnosis of rhabdomyosarcoma

    Tests and procedures used to diagnose rhabdomyosarcoma include: 

    • A health history, physical exam, and blood test to learn about symptoms, general health, past illnesses, and risk factors
    • Imaging tests, such as: 
      • An MRI to assess the primary tumor
      • A CT scan to see if rhabdomyosarcoma has spread to the lungs. If the tumor is in the lower part of the body, your child may need a CT scan of the abdomen and pelvis.
      • Full body imaging using bone scans or PET scans  to look for cancer in bone and other areas of the body
    • Bone marrow aspiration and biopsy to see if cancer has spread. 
    • A biopsy of the tumor 

    Your child may need other tests, depending on where the tumor is:

    •  Lymph node biopsy to see if rhabdomyosarcoma has spread to nearby lymph nodes
    • A lumbar puncture to look for cancer cells in the cerebrospinal fluid in tumors that arise in the head and neck region

    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 with tumor marked

    MRI of pediatric rhabdomyosarcoma patient's forearm

    MRI of pediatric rhabdomyosarcoma patient's abdomen with tumor marked

    MRI of rhabdomyosarcoma patient's abdomen

    MRI of pediatric rhabdomyosarcoma patient's abdomen from a cross section, or axial view.

    MRI of pediatric rhabdomyosarcoma patient's abdomen from a cross section, or axial, view

    Staging of rhabdomyosarcoma

    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:

    • Tumor location
    • Spread of disease
    • 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:

    1. Tumor (T) – Size and location of the primary tumor
    2. Node (N) – Spread of disease to lymph nodes
    3. Metastasis (M) – Spread of disease to other parts of the body

    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

    Treatment of rhabdomyosarcoma

    Rhabdomyosarcoma requires a combination of treatments. These can include:

    • Chemotherapy
    • Surgery
    • Radiation therapy

    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. 

    • Surgery will remove the tumor when possible. A small amount of tissue around the tumor and nearby lymph nodes is removed, too. 
    • Chemotherapy uses powerful medicines to kill cancer cells. Or it may stop them from growing. Most chemotherapy is given through injections. Sometimes it is given by mouth.
      • Chemotherapy before surgery is to help make the tumor smaller.
      • Chemotherapy after surgery is to kill any remaining cancer cells and to help keep the tumor from coming back.
    • Radiation therapy may be used to treat rhabdomyosarcoma in combination with chemotherapy and/or surgery. Radiation uses high energy particles to kill or stop the growth of cancer cells. Radiation therapy is the main treatment for metastatic disease in rhabdomyosarcoma.
    • Other treatments include: 

    Prognosis of rhabdomyosarcoma

    The chance of recovery from rhabdomyosarcoma depends on:

    • Size and location of the tumor
      • Larger tumors are more difficult to treat. These are tumors that are greater than 5 centimeters (cm).
      • Some tumors have a better outlook based on location. Tumors generally have a higher chance of cure when they are in the:
        • Eye area
        • Head and neck (except if they are near the membranes of the brain or spinal cord)
        • Genital or urinary tract (except in the bladder or prostate)
        • Bile ducts
    • Patient age (younger and older patients tend to do worse) 
    • Type of rhabdomyosarcoma: Embryonal tumors tend to have a better outcome than alveolar tumors.
    • Whether the cancer has spread to lymph nodes or other parts of the body
    • The ability of surgery to completely remove the tumor

    Risk groups

    Doctors classify rhabdomyosarcoma patients into risk groups to help plan treatments.

    • Low Risk – Patients with low-risk disease have an embryonal tumor that can be completely removed with surgery or a tumor around the eye that cannot be removed.
    • Intermediate Risk – Patients with intermediate or medium-risk disease have:
      • An embryonal tumor that is not completely removed with surgery
      • An alveolar tumor that has not spread to distant parts of the body.
    • High Risk – Patients with high-risk disease have cancer that has spread, or stage 4 disease.

    Risk classification helps doctors decide:

    • What treatments to use
    • Dose of medication
    • Number of treatments

     

    If rhabdomyosarcoma comes back, the prognosis may be affected by:

    • Where in the body the cancer came back
    • How much time passed between the original cancer and its return
    • What therapies were originally used to treat the cancer

    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.

  2. Non-rhabdomyosarcoma soft tissue sarcoma (NRSTS)

    What is non-rhabdomyosarcoma soft tissue sarcoma?

    A non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) is a tumor that develops in the body’s soft tissues. There are many types of these tumors. But they are usually treated in a similar way.

    NRSTS tumors account for about 4% of all childhood cancers. They are most common in teens.

    NRSTS can form anywhere in the body. But it is most often found in the arms or legs. An NRSTS can also occur in the head and neck, chest, abdomen, or pelvis.

    The most common types of NRSTS tumors in children include:

    • Fibrosarcoma
    • Synovial sarcoma 
    • Malignant peripheral nerve sheath tumors

    Other types of NRSTS tumors are:

    • Clear cell sarcoma
    • Epithelioid sarcoma
    • Hemangiopericytoma
    • Leiomyosarcoma
    • Liposarcoma
    • Malignant fibrous histiocytoma
    • Non-synovial cell sarcoma
    • Undifferentiated sarcoma

    Symptoms of NRSTS

    Symptoms of NRSTS depend on where the tumor is located. Often there are no symptoms in the early stages other than a painless lump.

    Problems such as pain or weakness may develop as the tumor grows and presses on other structures.

    More advanced disease can sometimes cause symptoms such as fever, sweating, or weight loss.

    Risk factors and causes of NRSTS

    NRSTS tumors in children are most often seen infants and teens. These tumors are slightly more common in males. Previous treatment with radiation therapy can also increase risk for NRSTS.

    Certain conditions passed through families can increase risk for NRSTS. These include:

    • Li-Fraumeni syndrome
    • Neurofibromatosis type 1
    • Familial adenomatous polyposis
    • Werner syndrome
    • Retinoblastoma 1 gene changes
    • SMARCB1 gene changes
    • Mismatch repair syndromes
    • Juvenile myofibromatosis type 1

    Diagnosis of NRSTS

    Several types of procedures and tests are used to diagnose NRSTS. These include:

    • A health history, physical exam, and blood tests to learn about symptoms, general health, past illnesses, and risk factors 
    • Imaging tests including MRI or CT scan of the primary tumor.
    • A biopsy of the tumor
    • A CT scan of the chest to see if cancer has spread to the lungs
    • Full-body imaging using bone scans or PET scans to look for cancer that has spread to bone and other areas of the body
    • Imaging and biopsy of lymph nodes  

    Staging of NRSTS

    Soft tissue sarcomas are classified by stage. Staging is based on features of the tumor such as:

    • Size
    • Whether it has spread
    • Grade of the tumor
       

    Soft tissue sarcomas can be grouped into 4 stages. The American Joint Committee on Cancer (AJCC) uses these guidelines:

    Stage Description
    		 Tumor Grade
    Stage IA The tumor is no larger than 5 centimeters (cm). It has not spread to lymph nodes or other parts of the body. 1
    Stage IB
    The tumor is larger than 5 cm across. It has not spread to lymph nodes or other parts of the body.
    		 1
    Stage IIA
    The tumor is no larger than 5 cm across. It has not spread to lymph nodes or other parts of the body.
    		 2 or 3
    Stage IIIA
    The tumor is 5-10 cm across. It has not spread to lymph nodes or other parts of the body.
    		 2 or 3
    Stage IIIB
    The tumor is greater than 10 cm across. It has not spread to lymph nodes or other parts of the body.
    		 2 or 3
    Stage IV
    The tumor is any size and any grade. It has spread to nearby lymph nodes and/or other parts of the body.
    		 Any

    Treatment of NRSTS

    Surgery

    Surgery is used to remove as much of the cancer as possible. Doctors also remove a small amount of tissue around the tumor and nearby lymph nodes to make sure that no cancer is left behind.

    Low-risk disease may be treated with surgery alone if the tumor can be fully removed with no remaining cancer cells.

    Radiation therapy

    Radiation therapy may be used to treat NRSTS that cannot be removed completely with surgery. In some cases, radiation may be used before surgery to make the tumor easier to remove. 

    Chemotherapy

    Chemotherapy uses powerful medicines to kill cancer cells or stop them from growing and making new cancer cells. Chemotherapy does not work well for most NRSTS patients.

    It is most often used to treat high-risk patients. These include patients whose disease has spread or those who cannot be treated with surgery.

    Targeted therapies

    Scientists are studying targeted therapies to treat NRSTS. These drugs act on specific targets of cancer cells to stop them from growing. Certain therapies are specific for subtypes of NRSTS. Many of these treatments are being studied in clinical trials.

    Prognosis for NRSTS

    The chance of recovery from NRSTS depends on:

    • Type of tumor
    • Where the tumor formed in the body
    • Tumor grade and how the cells look under a microscope (histology); low-grade tumors have a better outcome
    • Size of the tumor: Larger tumors (greater than 5 cm) are harder to treat
    • How deep the tumor is located below the skin
    • Whether the cancer has spread to lymph nodes or other parts of the body
    • Whether surgery can completely remove the tumor
    • Whether the tumor is new or has come back
    • Whether radiation has been used to treat the cancer

    Patients who have a tumor that meets certain conditions have a better prognosis:
     

      Favorable Unfavorable
    Tumor grade (histology)
    		 Low grade High grade
    Tumor size
    		 Less than 5 cm Greater then 5 cm
    Spread of disease (metastatic)
    		 No Yes
    Complete removal with surgery
    		 Yes No

    About 15% of patients have disease that has spread at diagnosis. The lung is the most common site for metastasis of NRSTS.

    It is rare, but NRSTS may spread to bone or lymph nodes.

    If the cancer has already spread at diagnosis, the chance of long-term survival is less than 20%.

    NRSTS that returns after treatment 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.

Sitara

Finding Strength in the Journey

Sitara Khan overcame cultural and gender barriers to receive the treatment she needed for her rhabdomyosarcoma.

Read Sitara's story

Life after soft tissue sarcoma

Monitoring for recurrence

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.

Health after cancer

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:

  • Heart problems
  • Kidney damage
  • Infertility
  • Endocrine problems
  • Second cancers

All survivors should continue to have regular physical checkups and screenings by a primary physician. A healthy lifestyle and eating habits can also help.

Questions to ask your child’s care team

  • What are our treatment options?
  • What are the possible side effects of each treatment?
  • What can be done to manage side effects?
  • Will my child need to be in the hospital for treatment?
  • Where is the treatment available? Is it close to home or will we have to travel?

Key points about soft tissue sarcoma

  • Soft tissue sarcoma is a type of cancer that grows in the body’s soft connective tissue.
  • There are 2 main types: Rhabdomyosarcoma and non-rhabdomyosarcoma soft tissue sarcoma (NRSTS).
  • Certain inherited conditions can increase risk for soft tissue sarcomas.
  • Treatment for soft tissue sarcoma usually includes surgery, chemotherapy, or radiation therapy.

—
Reviewed: December 2022

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