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Mesenchymal Chondrosarcoma of the Chest Wall

Abstract Mesenchymal chondrosarcoma (MC) is a very rare malignant mesenchymal neoplasm of the bone and soft tissue. It is composed of round to slightly spindled primitive mesenchymal cells arrayed about a prominent hemangiopericytoma-like vasculature, with production of relatively well-differentiated cartilage. The disease primarily affects young adults with a predilection for flat bones such as the sternum, rib cage, and craniofacial locations. In contrast to conventional chondrosarcoma, approximately one third of MC is extraskeletal. As the round cell component can be difficult to differentiate from other small round blue cell tumors such as Ewing sarcoma, small cell osteosarcoma, alveolar rhabdomyosarcoma, or lymphoma, the pathological evaluation of biopsy tissue, which lacks a cartilaginous component, may be challenging. This review addresses the clinical features, histopathology, differential diagnosis, and oncologic outcome of MC.