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


    Skeletal muscle tumors

    Skeletal muscle is attached to bones and helps the body move.

    • Rhabdomyosarcoma. Rhabdomyosarcoma is the most common childhood soft tissue sarcoma. See the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.

    Smooth muscle tumors

    Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus.

    • Leiomyosarcoma. This smooth muscle tumor has been linked with Epstein-Barr virus in children who also have HIV /AIDS. Leiomyosarcoma may also form as a second cancer in survivors of inherited retinoblastoma, even many years after their treatment for retinoblastoma.

    So-called fibrohistiocytic tumors

    So-called fibrohistiocytic tumors include the following types:

    • Plexiform fibrohistiocytic tumor. This is a rare tumor that usually affects children and young adults. The tumor usually starts as a painless growth on or just under the skin on the arm, hand, or wrist. It may rarely spread to nearby lymph nodes or to the lungs.
    • Undifferentiated pleomorphic sarcoma. This type of tumor may form in parts of the body where patients have received radiation therapy in the past, or as a second cancer in children with retinoblastoma. The tumor is usually found on the arms or legs and may spread to other parts of the body.

    Peripheral nervous system tumors

    • Malignant peripheral nerve sheath tumor. This is a tumor that forms in the protective covering of nerves that are not part of the brain or spinal cord. Some children who have a malignant peripheral nerve sheath tumor have a rare genetic condition called neurofibromatosis type 1 (NF1). This tumor may be low grade or high grade.

    Tumors of unknown origin

    Tumors of unknown origin (the place where the tumor first formed is not known) include the following types:

    • Alveolar soft part sarcoma . This is a rare tumor of the soft supporting tissue that connects and surrounds the organs and other tissues. It may grow slowly and has often spread to other parts of the body at the time of diagnosis. Alveolar soft part sarcoma may have a better prognosis when the tumor is 5 centimeters or smaller or when the tumor is completely removed by surgery.
    • Clear cell sarcoma of soft tissue. This is a soft tissue tumor that begins in a tendon (tough, fibrous, cord-like tissue that connects muscle to bone or to another part of the body). Clear cell sarcoma may spread to nearby lymph nodes.
    • Desmoplastic small round cell tumor. This tumor most often affects boys and may form and spread in the abdomen and pelvis or tissues around the testes. Desmoplastic small round cell tumor may also spread to the lungs and other parts of the body.
    • Epithelioid sarcoma. This is a rare sarcoma that usually starts deep in soft tissue as a slow growing, firm lump and may spread to the lymph nodes.
    • Perivascular epithelioid cell tumors (PEComas). Benign (not cancer) PEComas are commonly found in children with an inherited condition called tuberous sclerosis.
    • Extrarenal (extracranial) rhabdoid tumor. This is a rare, fast-growing tumor of soft tissues such as the liver and peritoneum. It usually occurs in young children, including newborns, but it can occur in older children and adults.
    • Extraskeletal myxoid chondrosarcoma. This is a rare soft tissue sarcoma that may be found in children and teenagers. This tumor may come back after treatment. Over time, it tends to spread to other parts of the body including the lymph nodes and the lungs.
    • Primitive neuroectodermal tumor (PNET)/extraskeletalEwing tumor. See the PDQ summary on Ewing Sarcoma Treatment for more information.
    • Synovial sarcoma. Synovial sarcoma is a common type of soft tissue sarcoma in children and teenagers. Synovial sarcoma usually forms in the tissues around the joints in the arms or legs, but may also form in the trunk, head, or neck. The cells of this tumor usually have a certain genetic change called a translocation (part of one chromosome switches places with part of another chromosome). Larger tumors have a greater risk of spreading to other parts of the body, including the lungs. Children younger than 10 years and those whose tumor is 5 centimeters or smaller have a better prognosis.
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