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Extraskeletal Myxoid Chondrosarcoma
Stephanie A. Bernard, MD; Catherine C. Roberts, MD; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Chordoid sarcoma, tendosynovial sarcoma
          • Definitions

            • Rare malignant soft tissue tumor of uncertain differentiation with hemorrhagic, multinodular appearance

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Nuclear Medicine Findings

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Staging, Grading, & Classification

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            • Molecular Genetics

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. Chiusole B et al: Extraskeletal myxoid chondrosarcoma: clinical and molecular characteristics and outcomes of patients treated at two institutions. Front Oncol. 10:828, 2020
                                      2. Baheti AD et al: Myxoid soft-tissue neoplasms: comprehensive update of the taxonomy and MRI features. AJR Am J Roentgenol. 204(2):374-85, 2015
                                      3. Oike N et al: Extraskeletal myxoid chondrosarcoma arising in the femoral vein: a case report. Skeletal Radiol. 43(10):1465-9, 2014
                                      4. Petscavage-Thomas JM et al: Soft-tissue myxomatous lesions: review of salient imaging features with pathologic comparison. Radiographics. 34(4):964-80, 2014
                                      5. Bhamra JS et al: Intra-articular extraskeletal myxoid chondrosarcoma of the ankle. Skeletal Radiol. 41(8):1017-20, 2012
                                      6. Weiss SW et al: Cartilaginous soft tissue tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 1023-31, 2008
                                      7. Ehara S et al: Skeletal recurrences and metastases of extraskeletal myxoid chondrosarcoma. Skeletal Radiol. 36(9):823-7, 2007
                                      8. Kransdorf MJ et al: Extraskeletal osseous and cartilaginous tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 470-2, 2006
                                      9. Tateishi U et al: MRI features of extraskeletal myxoid chondrosarcoma. Skeletal Radiol. 35(1):27-33, 2006
                                      10. Murphey MD et al: From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 23(5):1245-78, 2003
                                      11. Lucas DR et al: Extraskeletal myxoid chondrosarcoma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 213-5, 2002
                                      12. Ryan JM et al: Metastases to the liver from extraskeletal myxoid chondrosarcoma and successful treatment with percutaneous ethanol injection. Clin Radiol. 55(4):314-7, 2000
                                      13. Takeda A et al: Extraskeletal myxoid chondrosarcoma with multiple skeletal metastases. J Orthop Sci. 5(2):171-4, 2000
                                      14. Gebhardt MC et al: Extraskeletal myxoid chondrosarcoma of the knee. Skeletal Radiol. 28(6):354-8, 1999
                                      15. Sciot R et al: t(9;22)(q22-31;q11-12) is a consistent marker of extraskeletal myxoid chondrosarcoma: evaluation of three cases. Mod Pathol. 8(7):765-8, 1995
                                      16. Disler DG et al: Extensive skeletal metastases from chondrosarcoma without pulmonary involvement. Skeletal Radiol. 22(8):595-9, 1993
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Synonyms

                                                • Chordoid sarcoma, tendosynovial sarcoma
                                              • Definitions

                                                • Rare malignant soft tissue tumor of uncertain differentiation with hemorrhagic, multinodular appearance

                                              IMAGING

                                              • General Features

                                                • CT Findings

                                                  • MR Findings

                                                    • Nuclear Medicine Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          • Staging, Grading, & Classification

                                                            • Gross Pathologic & Surgical Features

                                                              • Microscopic Features

                                                                • Molecular Genetics

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. Chiusole B et al: Extraskeletal myxoid chondrosarcoma: clinical and molecular characteristics and outcomes of patients treated at two institutions. Front Oncol. 10:828, 2020
                                                                          2. Baheti AD et al: Myxoid soft-tissue neoplasms: comprehensive update of the taxonomy and MRI features. AJR Am J Roentgenol. 204(2):374-85, 2015
                                                                          3. Oike N et al: Extraskeletal myxoid chondrosarcoma arising in the femoral vein: a case report. Skeletal Radiol. 43(10):1465-9, 2014
                                                                          4. Petscavage-Thomas JM et al: Soft-tissue myxomatous lesions: review of salient imaging features with pathologic comparison. Radiographics. 34(4):964-80, 2014
                                                                          5. Bhamra JS et al: Intra-articular extraskeletal myxoid chondrosarcoma of the ankle. Skeletal Radiol. 41(8):1017-20, 2012
                                                                          6. Weiss SW et al: Cartilaginous soft tissue tumors. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 1023-31, 2008
                                                                          7. Ehara S et al: Skeletal recurrences and metastases of extraskeletal myxoid chondrosarcoma. Skeletal Radiol. 36(9):823-7, 2007
                                                                          8. Kransdorf MJ et al: Extraskeletal osseous and cartilaginous tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 470-2, 2006
                                                                          9. Tateishi U et al: MRI features of extraskeletal myxoid chondrosarcoma. Skeletal Radiol. 35(1):27-33, 2006
                                                                          10. Murphey MD et al: From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 23(5):1245-78, 2003
                                                                          11. Lucas DR et al: Extraskeletal myxoid chondrosarcoma. In Fletcher CDM et al: World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. IARC Press. 213-5, 2002
                                                                          12. Ryan JM et al: Metastases to the liver from extraskeletal myxoid chondrosarcoma and successful treatment with percutaneous ethanol injection. Clin Radiol. 55(4):314-7, 2000
                                                                          13. Takeda A et al: Extraskeletal myxoid chondrosarcoma with multiple skeletal metastases. J Orthop Sci. 5(2):171-4, 2000
                                                                          14. Gebhardt MC et al: Extraskeletal myxoid chondrosarcoma of the knee. Skeletal Radiol. 28(6):354-8, 1999
                                                                          15. Sciot R et al: t(9;22)(q22-31;q11-12) is a consistent marker of extraskeletal myxoid chondrosarcoma: evaluation of three cases. Mod Pathol. 8(7):765-8, 1995
                                                                          16. Disler DG et al: Extensive skeletal metastases from chondrosarcoma without pulmonary involvement. Skeletal Radiol. 22(8):595-9, 1993