• Soft-tissue Sarcoma Overview

    Soft-tissue sarcomas are cancerous tumors that being in tissues that connect, support or surround organs and other body tissue—muscle, tendon, synovial tissue (around joints), fat, blood vessels, lymph vessels, and nerves. They can develop almost anywhere in the body, but in children are most common in the arms, legs and trunk (chest and abdomen).

    While the cause of soft-tissue sarcomas is not well understood, genetics may play a role in these diseases. Some inherited diseases, such as Li Fraumeni syndrome and neurofibromatosis type 1, increase the risk.

    Soft-tissue Sarcoma Treatment at Dana-Farber/Boston Children's

    Patients with soft-tissue sarcomas are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Bone & Soft Tissue Program. Continue reading to learn more about soft-tissue sarcomas or visit the Bone & Soft Tissue Program homepage to learn about our expertise.

    Types of Soft Tissue Sarcomas

    There are many different types of soft-tissue sarcomas, including:

    • Adamantinoma – a cancerous tumor found most often in the shin bone (tibia) and jaw bone (mandible).
    • Alveolar soft part sarcoma – a tumor that starts in connective tissue, including fat, muscle and nerve.
    • Desmoid tumors – tumors that form in fibrous tissue that makes up tendons and ligaments.
    • Ewing sarcoma – a cancer that grows in bone or soft tissue around bone.
    • Fibrosarcoma – a tumor that originates in connective tissue at the end of bones in the arms or legs and spreads to surrounding soft tissue.
    • Infantile fibrosarcoma – tumors that develop in tissue that forms tendons and ligaments and that has a unique behavior in the first few months of life.
    • Leiomyosarcoma – tumors that develop in involuntary muscle tissue, usually in the abdomen, bowel, uterus and blood vessels.
    • Liposarcoma – tumors that develop in fat tissue, most often in the abdominal cavity.
    • Malignant fibrous histiocytoma – a tumor that starts in either bone or, more often, in the soft tissue that connects, supports and surrounds organs and body parts.
    • Peripheral nerve sheath tumor – tumors that form in cells that surround nerves.
    • Rhabdomyosarcoma – the most common soft-tissue sarcoma, which begins in skeletal muscles.
    • Synovial sarcoma – malignant tumors in soft tissues around joints, most commonly the knee.

    Symptoms & Diagnosis

    Soft-tissue sarcomas affect tissue that is elastic and easily moved, which means that a tumor may exist for a long time before being discovered, growing larger and pushing aside surrounding tissue.

    While each disease has its own specific symptoms, the most common symptoms of soft-tissue sarcoma include:

    • Painless swelling or a lump
    • Pain or soreness caused by compressed nerves or muscles
    • Limping or other difficulty using the legs and feet

    The diagnostic procedures used to determine the exact type of soft-tissue sarcoma a child has, and whether it has spread, include:

    • Physical exam
    • X-ray
    • Computerized tomography (CT or CAT) scan
    • Magnetic resonance imaging (MRI) scan
    • Biopsy of tissue to provide definitive information regarding tumor type
    • Bone scan or PET scan
    • Complete blood count
    • Blood chemistry tests

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Treatment for soft-tissue sarcomas depends on the type of tumor a child has. It may involve a combination of:

    • Surgery – including limb-salvage surgery to remove the portion of a limb where the tumor is located or amputation if the tumor cannot be removed
    • Chemotherapy 
    • Radiation therapy 

    Progressive or Recurrent Disease

    Depending on the type of tumor, treatment for recurrent or progressive disease may include watchful waiting, surgery followed by radiation therapy, or a combination of chemotherapy drugs. Treatment decisions are based on the site of the recurrence, prior therapies, and individual patient considerations.

    If a child has a progressive or recurrent tumor, he/she may be eligible for a number of experimental therapies available through the Children’s Oncology Group. The prognosis for progressive or recurrent soft-tissue sarcomas is poor.

    Long-term Outlook

    Over the past 30 years, there has been drastic improvement in survival for children with soft-tissue sarcomas. Complete surgical removal can control the tumor in about 80 percent of cases. Adding radiation therapy after surgery can increase cure rates to 90 to 95 percent. Incomplete tumor removal and spreading to other parts of the body gives a poorer prognosis. The best results are achieved with smaller, less aggressive and more localized tumors.
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