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While the most common form of soft-tissue sarcoma affecting children and adolescents is rhabdomyosarcoma, there are many other types of this rare cancer. Researchers at St. Jude Children’s Research Hospital recently evaluated the treatment outcomes for young persons with non-rhabdomyosarcoma soft-tissue sarcoma.

Soft-tissue sarcoma, a rare cancer in children and adolescents, is characterized by the presence of cancer cells in the soft tissues of the body. The cancer can begin growing in a number of soft tissue types, including muscles, tendons, connective tissue (called

fibrous tissue), fat, blood vessels, nerves, and the tissue around the joints (called

synovial tissue). The cancer can develop in any part of the body, but in children and adolescents, it most often affects the arms, legs, or trunk area. There are many different types of childhood soft-tissue sarcomas, with rhabdomyosarcoma being the most common. Non-rhabdomyosarcoma soft-tissue sarcomas include synovial sarcoma, fibrous histiocytoma, fibrosarcoma, nerve sheath tumor, alveolar soft-part sarcoma, leiomyosarcoma, epithelioid sarcoma, and other types.

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Treatment options for children and adolescents with soft-tissue sarcoma depend on the specific location, type, and stage of disease (extent of cancer at diagnosis). Usually, individuals with soft-tissue sarcoma first undergo surgery to remove as much of the cancer as possible. After surgery, they often receive an intensive combination of chemotherapy and radiation therapy to kill any remaining cancer cells and to help prevent a

recurrence (return) of the cancer later. Because non-rhabdomyosarcoma soft-tissue sarcomas are so rare in young persons, it is especially important for researchers to evaluate and share the treatment outcomes in these cases. In doing so, new treatments and treatment strategies can be developed and studied, resulting in more effective options for children and adolescents with soft-tissue sarcoma.

Researchers at St. Jude Children’s Research Hospital evaluated the treatment outcomes of 121 children and adolescents who were seen consecutively between 1969 and 1996. All were treated for soft-tissue sarcoma of the non-rhabdomyosarcoma type. An average of 9 years after treatment, the overall survival rate was 89% and the survival rate without any recurrence was 77%. The researchers’ analysis showed that the outcomes were worse in patients who had surgery that was not extensive enough, large-sized cancer, cancer in the abdomen, or cancer that was of a high-grade (or quickly growing) type. In addition, the results revealed that those who did not have radiation therapy had a higher incidence of recurrence of the cancer after treatment.

These findings are useful for researchers who are treating children and adolescents with non-rhabdomyosarcoma soft-tissue sarcomas. In particular, a review of the outcomes for patients with each specific type of soft-tissue sarcoma may allow researchers to determine which patients are at high or low risk for recurrence and to adjust their treatment regimens accordingly. Parents of children or adolescents with soft-tissue sarcoma may wish to talk with their doctor about participating in a clinical trial (research study) in which promising new chemotherapy/radiation therapy treatments are being studied. (Journal of Clinical Oncology, Vol 17, No 12, pp 3697-3705, 1999)

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