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Treatment of Newly Diagnosed Childhood Soft Tissue Sarcoma

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    Treatment options under clinical evaluation for alveolar soft part sarcoma

    The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

    • NCT00942877 (Phase II Study of Cediranib in Patients With Alveolar Soft Part Sarcoma): This phase II study of cediranib in patients with alveolar soft part sarcoma is being conducted at the Clinical Center of the National Institutes of Health. Patients aged 18 years and older are eligible to participate.

    Clear cell sarcoma of soft tissue

    Clear cell sarcoma (formerly and inappropriately called malignant melanoma of soft parts), also called clear cell sarcoma of tendons and aponeuroses, is somewhat similar to cutaneous malignant melanoma but is cytogenetically distinct; most cases have a t(12;22)(q13;q12) translocation that has not been reported in melanoma.[84] In one series, clear cell sarcoma demonstrated a propensity to metastasize to regional lymph nodes (12%–43%).[85]

    Patients who have small, localized tumors with low mitotic rate and intermediate histologic grade fare best.[86]

    The primary treatment for clear cell sarcoma is complete surgical resection, with the addition of radiation therapy for uncertain or involved margins. Chemotherapy is rarely effective.[87]

    Desmoplastic small round cell tumor

    Desmoplastic small round cell tumor is a primitive sarcoma that most frequently involves the abdomen, pelvis, or tissues around the testes.[88,89,90] The tumor occurs more commonly in males and invades locally but may spread to the lungs and elsewhere.

    Cytogenetic studies of these tumors have demonstrated the recurrent translocation t(11;22)(p13;q12), which has been characterized as a fusion of the WT1 and EWS genes.[91]

    Treatment

    There is no standard approach to the treatment of desmoplastic small round cell tumor. Complete surgical resections are rare, and the overall prognosis for desmoplastic small round cell tumor remains extremely poor, with reported rates of death at 90%. A small series of patients who were treated with hyperthermic intraperitoneal chemotherapy with acceptable toxicity has been reported.[92]

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