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Extraskeletal Mesenchymal Chondrosarcoma
Eric Walker, MD, MHA, FACR, FACHE; Catherine C. Roberts, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • High-grade, malignant, biphasic, primitive mesenchymal tumor containing well-differentiated, organized hyaline cartilage component
              • ~ 40% of cases affect somatic soft tissue
              • Histologic types of lesions that account for extraskeletal chondrosarcoma are myxoid chondrosarcoma, mesenchymal chondrosarcoma, and, very rarely, low-grade chondrosarcoma

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Fanburg-Smith JC et al: Mesenchymal chondrosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press, 2020
                                      2. Kao YC et al: What is new about the molecular genetics in matrix-producing soft tissue tumors? The contributions to pathogenetic understanding and diagnostic classification. Virchows Arch. 476(1):121-34, 2020
                                      3. Stacchiotti S et al: Extraskeletal myxoid chondrosarcoma: state of the art and current research on biology and clinical management. Cancers (Basel). 12(9), 2020
                                      4. Gupta SR et al: A rare case of extraskeletal mesenchymal chondrosarcoma with dedifferentiation arising from the buccal space in a young male. J Maxillofac Oral Surg. 14(Suppl 1):293-9, 2015
                                      5. Kransdorf MJ et al: Extraskeletal osseous and cartilaginous lesions. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 580-3, 2014
                                      6. Herrera A et al: Primary orbital mesenchymal chondrosarcoma: case report and review of the literature. Case Rep Med. 2012:292147, 2012
                                      7. Weiss SW et al: Cartilaginous soft tissue tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 1031-6, 2008
                                      8. Anderson JT et al: Extraskeletal mesenchymal chondrosarcoma of the forearm: a case report. J Hand Surg Am. 32(3):389-92, 2007
                                      9. Pellitteri PK et al: Mesenchymal chondrosarcoma of the head and neck. Oral Oncol. 43(10):970-5, 2007
                                      10. Hashimoto N et al: Extraskeletal mesenchymal chondrosarcoma: an imaging review of ten new patients. Skeletal Radiol. 34(12):785-92, 2005
                                      11. Murphey MD et al. Imaging of primary chondrosarcoma: radiologic-pathologic correlation. RadioGraphics 23:1245-78. 2003
                                      12. Shapeero LG et al: Extraskeletal mesenchymal chondrosarcoma. Radiology. 186(3):819-26, 1993
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Definitions

                                                  • High-grade, malignant, biphasic, primitive mesenchymal tumor containing well-differentiated, organized hyaline cartilage component
                                                  • ~ 40% of cases affect somatic soft tissue
                                                  • Histologic types of lesions that account for extraskeletal chondrosarcoma are myxoid chondrosarcoma, mesenchymal chondrosarcoma, and, very rarely, low-grade chondrosarcoma

                                                IMAGING

                                                • General Features

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Angiographic Findings

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Fanburg-Smith JC et al: Mesenchymal chondrosarcoma. In Fletcher CDM et al: WHO Classification of Tumours: Soft Tissue and Bone Tumours. 5th ed. IARC Press, 2020
                                                                          2. Kao YC et al: What is new about the molecular genetics in matrix-producing soft tissue tumors? The contributions to pathogenetic understanding and diagnostic classification. Virchows Arch. 476(1):121-34, 2020
                                                                          3. Stacchiotti S et al: Extraskeletal myxoid chondrosarcoma: state of the art and current research on biology and clinical management. Cancers (Basel). 12(9), 2020
                                                                          4. Gupta SR et al: A rare case of extraskeletal mesenchymal chondrosarcoma with dedifferentiation arising from the buccal space in a young male. J Maxillofac Oral Surg. 14(Suppl 1):293-9, 2015
                                                                          5. Kransdorf MJ et al: Extraskeletal osseous and cartilaginous lesions. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 3rd ed. Lippincott Williams & Wilkins. 580-3, 2014
                                                                          6. Herrera A et al: Primary orbital mesenchymal chondrosarcoma: case report and review of the literature. Case Rep Med. 2012:292147, 2012
                                                                          7. Weiss SW et al: Cartilaginous soft tissue tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 1031-6, 2008
                                                                          8. Anderson JT et al: Extraskeletal mesenchymal chondrosarcoma of the forearm: a case report. J Hand Surg Am. 32(3):389-92, 2007
                                                                          9. Pellitteri PK et al: Mesenchymal chondrosarcoma of the head and neck. Oral Oncol. 43(10):970-5, 2007
                                                                          10. Hashimoto N et al: Extraskeletal mesenchymal chondrosarcoma: an imaging review of ten new patients. Skeletal Radiol. 34(12):785-92, 2005
                                                                          11. Murphey MD et al. Imaging of primary chondrosarcoma: radiologic-pathologic correlation. RadioGraphics 23:1245-78. 2003
                                                                          12. Shapeero LG et al: Extraskeletal mesenchymal chondrosarcoma. Radiology. 186(3):819-26, 1993