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    Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Patient Information [NCI] - General Information About Childhood Soft Tissue Sarcoma


    Blood vessel tumors

    Blood vessel tumors include the following types:

    • Angiosarcoma (deep). Angiosarcomas may form inside blood vessels or lymph vessels. This type of tumor usually grows quickly.
    • Epithelioid hemangioendothelioma. In infants, these tumors often form in the liver but may form in other parts of the body such as the lung. They are usually benign (not cancer). Children with consumptive coagulopathy (also known as Kasabach-Merritt syndrome) may have an increased chance of epithelioid hemangioendothelioma. In children younger than 1 year, the tumor may go away without treatment. A small number of children may develop cancerous tumors over time. Infants with hemangiomas should be checked regularly with ultrasound exams and may be treated with a liver transplant if their tumors are benign. In some cases, the benign tumor may become malignant (cancer) and spread to the lungs, lymph nodes, bones, abdomen, or pelvis.
    • Hemangiopericytoma (infantile). This soft tissue tumor has many blood vessels. Children younger than 1 year have a better prognosis. In patients older than 1 year, this tumor is more likely to spread to distant tissues, including the lymph nodes and lungs.

    See the following PDQ summaries for information about types of soft tissue sarcoma not included in this summary:

    Soft tissue sarcoma occurs in children and adults.

    Soft tissue sarcoma in children may respond differently to treatment, and may have a better prognosis than soft tissue sarcoma in adults. (See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information on treatment in adults.)

    Having certain diseases and inherited disorders can increase the risk of childhood soft tissue sarcoma.

    Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your child's doctor if you think your child may be at risk.

    Risk factors for childhood soft tissue sarcoma include having the following inherited disorders:

    • Li-Fraumeni syndrome.
    • Neurofibromatosis type 1 (NF1).
    • Familial adenomatous polyposis (FAP).
    • Werner syndrome.
    • Retinoblastoma gene changes.
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