Abstract

Extremity sarcomas are a heterogeneous group of malignant tumors with a varied propensity for local recurrence. This review focuses on factors associated with local recurrence and survival, diagnostic workup, management, outcomes, and surveillance. Local recurrence is more common in patients with previous local recurrence, positive margins, high-grade histology, and deep tumors. In the absence of metastases, the mainstay of treatment is limb-sparing surgery, with radiation to improve local control. Modalities such as brachytherapy or proton therapy may be valuable in the setting of previous irradiation. Systemic chemotherapy is typically limited to the treatment of distant disease, although chemotherapy can be delivered locally via limb perfusion or infusion for locally advanced or recurrent disease. Amputation is used if local control cannot be achieved while preserving adequate limb function or as a palliative option for pain, bleeding, or fungating tumors. Prognostic factors associated with poor survival include tumors that recur with high-grade histology, with a large size (> 5 cm), or within a short interval (< 16 months). Reports of 5-year overall survival in patients with locally recurrent sarcomas vary from 36 to 65%. Surveillance includes physical examination, cross-sectional imaging, and chest x-ray. Genetic profiling and intratumoral injections provide novel therapeutic targets. This review contains 1 figure, 4 tables and 40 references. Key words: chemotherapy, hyperthermic isolated limb perfusion, intratumoral injection, isolated limb perfusion, local recurrence, margin status, radiation, soft tissue sarcoma, wide local excision

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