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Current Approach to Pediatric Soft Tissue Sarcomas

Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

Key Words. Sarcoma • Soft tissue • Pediatric malignancy • Chemotherapy • Ewing’s sarcoma • Synovial sarcoma

Correspondence: Correspondence: Melinda Merchant, M.D., Ph.D., Pediatric Oncology Branch/NCI, 10 Center Drive, Building 10, Room 1W-3940, Bethesda, Maryland 20892, USA. Telephone: 301-443-7955; Fax: 301-451-7052; e-mail: merchanm{at}mail.nih.gov

Received July 17, 2009; accepted for publication October 6, 2009.

Disclosures: Melinda S. Merchant: None; Crystal L. Mackall: None.

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. No financial relationships relevant to the content of this article have been disclosed by the authors or independent peer reviewers.

The development of a new soft tissue lesion in an otherwise healthy child, adolescent, or young adult can present many challenges for pediatric or medical oncology teams. Although uncommon, the diagnosis of a soft tissue malignancy should always be considered in the differential diagnosis of persistent pain, even if no mass is palpable. The definitive diagnosis and treatment of a soft tissue mass is aided by timely scans, appropriate biopsy for anatomic and molecular pathology, and a treatment approach guided by the specific diagnosis. Because pediatric soft tissue sarcomas are rare, cooperative groups play a crucial role in defining the standard of care through retrospective series and well-designed prospective clinical trials. Enrollment of newly diagnosed patients in clinical studies should be encouraged in order to continue to improve outcomes and understanding of these rare tumors. This review focuses on the current recommendations for management of sarcomas that typically occur in the soft tissues of pediatric and young adult patients.