Soft-tissue sarcomas of the upper extremity are extremely rare, particularly within the pediatric population. Optimal management is heavily dependent upon early recognition, proper diagnostic evaluation, and referral to a pediatric sarcoma center for both tissue biopsy and definitive management. Cure is most likely realized in the setting of truly localized disease, where complete surgical resection can be accomplished. Despite advances in adjuvant therapy, surgery remains the most efficacious treatment modality for localized soft-tissue sarcomas, with radiation augmenting local control primarily for large high-grade lesions. Adherence to fundamental sarcoma surgery principles is critical to oncologic outcomes and should take priority over functional results. A thorough understanding of upper extremity compartmental anatomy is necessary in order to plan and execute complete tumor extirpation. The role for chemotherapy continues to evolve, though adjuvant administration for large high-grade tumors and palliative administration for metastatic disease are common. While over 50 types of soft-tissue sarcoma have been described, some of the more commonly encountered pediatric soft-tissue sarcomas of the upper extremity include alveolar rhabdomyosarcoma, epithelioid sarcoma, synovial sarcoma, infantile fibrosarcoma, dermatofibrosarcoma protuberans, and malignant peripheral nerve sheath tumors.
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