Childhood Soft Tissue Sarcoma

This includes fat, cartilage, connective tissue, muscles, skin, nerves, tendons, joints, blood vessels and lymph vessels.

The most common locations of soft tissue sarcomas are the arms and legs, but they can also grow in the head and neck area, chest, abdomen and pelvis. Soft tissue sarcoma can occur in children and adults. This information focuses on soft tissue sarcoma in children.

Types of childhood soft tissue sarcoma

Rhabdomyosarcoma (RMS) is the most common childhood soft tissue sarcoma, with a few hundred cases diagnosed in the United States each year. It grows in muscles attached to the skeleton.

Other childhood soft tissue tumors are sometimes called non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). Together, this group of different cancers makes up about 4% of pediatric cancers. They are classified by the part of the body in which they start and by the type of cells they contain.

Other than rhabdomyosarcoma, the most common soft tissue sarcomas in children include:

• Fat tissue tumors: Liposarcoma in children is usually low or intermediate grade and grows slowly. The most common type is myxoid liposarcoma.
• Connective tissue tumors:
  • Desmoid tumors (aggressive fibromatosis), which usually grow slowly and are unlikely to spread. Children with familial adenomatous polyposis (FAP) are at higher risk.
  • Fibrosarcoma often occurs in children under age 4, especially infants. Tumors tend to grow quickly but usually do not spread.
• Muscle tumors other than RMS: Leiomyosarcoma is associated with Epstein-Barr virus in children with HIV/AIDS. Survivors of retinoblastoma are also at higher risk.
• Skin tumors:
  • Plexiform fibrohistiocytic tumors begin as painless masses on or under the skin, usually in the arm, hand, or wrist.
  • Undifferentiated pleomorphic sarcoma can develop in children who have had radiation therapy or retinoblastoma.
• Nerve tumors: Malignant peripheral nerve sheath tumors grow in the covering of nerves outside the brain and spinal cord. Children with neurofibromatosis type 1 (NF1) are at higher risk.
• Tumors of unknown origin: Synovial sarcoma can develop in different parts of the body, most often near joints in the arms or legs, but also in the head, neck or trunk. Despite its name, it does not arise from synovial (joint) tissue. Instead, it is called “synovial” sarcoma because, under a microscope, the tumor cells can resemble cells found in joint linings. However, the exact type of normal cell these tumors come from is still unknown.
• Blood vessel tumors:
  • Angiosarcomas, which grow quickly in lymph or blood vessels.
  • Epithelioid hemangioendotheliomas, which usually develop in the liver in infants and may grow slowly.

Who gets childhood soft tissue sarcoma

Soft tissue sarcoma can occur at any age, but certain groups are at higher risk, including:

• Teenagers and infants under 1 year of age
• People with certain genetic conditions, such as neurofibromatosis type 1 (NF1), Li-Fraumeni syndrome, inherited retinoblastoma, Werner syndrome, familial adenomatous polyposis (FAP)
• Individuals with HIV/AIDS or Epstein-Barr virus

Having a risk factor does not mean a child will develop soft tissue sarcoma. However, it is important to discuss family and personal medical history with your child’s doctor. Genetic counseling and testing may be recommended.

Childhood soft tissue sarcoma symptoms

Soft tissue sarcoma often presents as a firm painless lump that steadily increases in size and may be fixed in place. Other symptoms vary depending on the type and location of the tumor.

They may include:

• Pain or tenderness, sometimes worsening at night
• Fatigue
• Difficulty moving a limb if near a joint
• Unexplained fever or weight loss

These symptoms do not always indicate soft tissue sarcoma, but they should be evaluated by a doctor.

Childhood soft tissue sarcoma diagnosis

A thorough work-up is essential for successful treatment. Tests may include:

• Imaging: CT scans, MRI scans, PET scans, X-rays and bone scans may be used to assess the tumor’s location and determine whether it has spread.
• Biopsy: A biopsy is the only way to confirm that a tumor is cancer. It may be performed using a needle or through an open surgical procedure.
• Bone marrow aspiration or biopsy and blood tests: These tests may be done in selected cases.
• Genetic testing: Genetic testing may be recommended if there is a suspicion of inherited cancer risk.

Childhood soft tissue sarcoma treatment

Treatment is tailored to each child and may include:

• Surgery: Removal of the tumor while sparing surrounding healthy tissue. Limb-sparing procedures are often possible.
• Chemotherapy: Often the first step to treat micrometastases not visible on imaging and to treat or shrink the primary tumor prior to surgery and/or radiation.
• Radiation therapy: Used to destroy remaining cancer cells or when surgery is not possible. Advanced techniques minimize the impact on growing bodies.
• Targeted therapy: Innovative agents designed to treat cancer based on its genetic or molecular profile.
• Clinical trials: Access to national and international research protocols may be recommended.

Childhood soft tissue sarcoma survival rates

Every child’s situation is unique, and outcomes can vary based on factors like the type of tumor, where it is located, how advanced it is at diagnosis and your child’s age.

Some types, such as rhabdomyosarcoma, often respond well to treatment, especially when found early and cared for by an experienced team. Other soft tissue sarcomas can behave differently, and outcomes may vary depending on the specific tumor type and stage.

Because there are so many factors involved, your child’s care team is the best source of information about what to expect. They can help you understand your child’s specific diagnosis, treatment plan and outlook, and answer any questions along the way.

Living with childhood soft tissue sarcoma

Supportive care is essential for recovery and quality of life:

• Physical therapy and rehabilitation after surgery
• Psychosocial support for children and families
• Regular follow-up to monitor for recurrence or long-term treatment effects
• Coordination of care among pediatric oncology specialists

Risk factors

A risk factor increases the chance of developing a certain disease. Risk factors for childhood soft tissue sarcomas include:

• Inherited genetic conditions: NF1, Li-Fraumeni syndrome, inherited retinoblastoma, Werner syndrome
• Familial adenomatous polyposis (FAP)
• Viral infections: HIV/AIDS and Epstein-Barr virus
• Age: Teenagers and infants under 1 year of age

Are soft tissue sarcomas hereditary?

Some soft tissue tumors can be inherited through passed-down genetic mutations. This accounts for approximately 20% of cases. Genetic counseling may be recommended. Learn more about the risk to you and your family on our genetic testing page.

Learn more about childhood soft tissue sarcoma:

• Childhood soft tissue sarcoma symptoms
• Childhood soft tissue sarcoma diagnosis
• Childhood soft tissue sarcoma treatment

Why choose UT MD Anderson for your childhood soft tissue sarcoma treatment?

At UT MD Anderson's Children's Cancer Hospital, we know your child's health and well being are your number one concern. Our renowned experts customize your child's care for soft tissue sarcoma, utilizing the most advanced treatments and techniques with the least impact on your child's growing body.

Because soft tissue sarcomas are rare, most physicians have little or no experience in diagnosing and treating them. As part of one of the world's most active cancer centers, Children's Cancer Hospital has remarkable experience and skill in these types of cancer. This can make a difference in your child's outcome.

Customized care

A team of specially trained physicians follows your child throughout treatment, all the way to survivorship. They communicate closely with each other, and with you, to ensure comprehensive, personalized care. They are supported by full complement of health care professionals dedicated to your child's treatment, including nurses, physician assistants, therapists and others.

Children's Cancer Hospital offers clinical trials for innovative new treatments for soft tissue sarcoma. Behind the scenes we are working on groundbreaking basic science research to change the future of pediatric cancer.

Treating the whole child

Children's Cancer Hospital is designed just for children, with a full range of services and amenities that help make the child and family's experience as comfortable as possible. We go beyond medical care to deliver a comprehensive experience that treats the whole child.

And at Children's Cancer Hospital, your child benefits from the resources and expertise of one of the nation's top cancer centers.