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Extraskeletal myxoid chondrosarcoma (EMCS) is a rare malignant soft tissue tumor that usually develops in the deep parts of the proximal extremities and limb girdles in middle-aged adults. Its biologic behavior and pathogenesis are poorly understood. EMCS has a prolonged and indolent clinical course, with a high rate of local recurrences and distant metastases before tumor-related death. The diagnosis and management of this tumor must be early and multidisciplinary.