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Chondrosarcoma
Behrang Amini, MD, PhD
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KEY FACTS

  • Terminology

    • Imaging

      TERMINOLOGY

      • Synonyms

        • Primary chondrosarcoma (CS), conventional CS, secondary CS, low-grade peripheral CS
      • Definitions

        • 4 subtypes defined by location and grade
        • Location
          • Central: within medullary cavity
          • Secondary peripheral: arising within cartilage cap of osteochondroma (OC)
            • Lesions arising from enchondroma not considered secondary under WHO classification
        • Grade
          • Atypical cartilaginous tumor (ACT)/CS, grade 1 (CS1): locally aggressive, hyaline cartilage-producing neoplasm
            • ACT: lesions in appendicular skeleton (long and short tubular bones)
            • CS1: lesions in axial skeleton (flat bones: pelvis, scapula, skull base)
          • CS2 (intermediate grade) and CS3 (high grade): malignant cartilage matrix-producing neoplasms
            • Grouped together

      IMAGING

      • General Features

        • Image-Guided Biopsy

          • Imaging Recommendations

            DIFFERENTIAL DIAGNOSIS

              PATHOLOGY

              • General Features

                • Staging, Grading, & Classification

                  • Gross Pathologic & Surgical Features

                    • Microscopic Features

                      CLINICAL ISSUES

                      • Presentation

                        • Demographics

                          • Natural History & Prognosis

                            • Treatment

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                Selected References

                                1. Lee S et al: Assessment of central cartilaginous tumor of the appendicular bone: inter-observer and intermodality agreement and comparison of diagnostic performance of CT and MRI. Acta Radiol. 63(3):376-86, 2022
                                2. Haygood TM et al: Chondroid tumors of bone. Semin Ultrasound CT MR. 42(2):123-33, 2021
                                3. Bovée JVMG et al: Central atypical cartilaginous tumour/chondrosarcoma, grade 1. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 370-2, 2020
                                4. Bovée JVMG et al: Central chondrosarcoma, grades 2 and 3. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 375-8, 2020
                                5. Bovée JVMG et al: Secondary peripheral atypical cartilaginous tumour/chondrosarcoma, grade 1. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 373-4, 2020
                                6. Bovée JVMG et al: Secondary peripheral chondrosarcoma, grades 2 and 3. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 379-80, 2020
                                7. Davies AM et al: A retrospective validation of an imaging protocol for the management of solitary central cartilage tumours of the proximal humerus and around the knee. Clin Radiol. 74(12):962-71, 2019
                                8. Subhawong TK et al: F-18 FDG PET differentiation of benign from malignant chondroid neoplasms: a systematic review of the literature. Skeletal Radiol. 46(9):1233-9, 2017
                                9. Ferrer-Santacreu EM et al: Enchondroma versus chondrosarcoma in long bones of appendicular skeleton: clinical and radiological criteria-A follow-Up. J Oncol. 2016:8262079, 2016
                                10. Brown MT et al: How safe is curettage of low-grade cartilaginous neoplasms diagnosed by imaging with or without pre-operative needle biopsy? Bone Joint J. 96-B(8):1098-105, 2014
                                11. Douis H et al: MRI differentiation of low-grade from high-grade appendicular chondrosarcoma. Eur Radiol. 24(1):232-40, 2014
                                12. van Maldegem AM et al: Comprehensive analysis of published studies involving systemic treatment for chondrosarcoma of bone between 2000 and 2013. Clin Sarcoma Res. 4:11, 2014
                                13. van Maldegem AM et al: Outcome of advanced, unresectable conventional central chondrosarcoma. Cancer. 120(20):3159-64, 2014
                                14. De Coninck T et al: Dynamic contrast-enhanced MR imaging for differentiation between enchondroma and chondrosarcoma. Eur Radiol. 23(11):3140-52, 2013
                                15. Italiano A et al: Advanced chondrosarcomas: role of chemotherapy and survival. Ann Oncol. 24(11):2916-22, 2013
                                16. Meftah M et al: Long-term results of intralesional curettage and cryosurgery for treatment of low-grade chondrosarcoma. J Bone Joint Surg Am. 95(15):1358-64, 2013
                                17. Bui KL et al: Cortical scalloping and cortical penetration by small eccentric chondroid lesions in the long tubular bones: not a sign of malignancy? Skeletal Radiol. 38(8):791-6, 2009
                                18. Giuffrida AY et al: Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database. J Bone Joint Surg Am. 91(5):1063-72, 2009
                                19. Hallor KH et al: Genomic profiling of chondrosarcoma: chromosomal patterns in central and peripheral tumors. Clin Cancer Res. 15(8):2685-94, 2009
                                20. Puri A et al: Chondrosarcoma of bone: does the size of the tumor, the presence of a pathologic fracture, or prior intervention have an impact on local control and survival? J Cancer Res Ther. 5(1):14-9, 2009
                                21. Murphey MD et al: From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 23(5):1245-78, 2003
                                22. Ollivier L et al: Imaging of chondrosarcomas. Cancer Imaging. 4(1):36-8, 2003
                                23. Geirnaerdt MJ et al: Cartilaginous tumors: fast contrast-enhanced MR imaging. Radiology. 214(2):539-46, 2000
                                24. Lee FY et al: Chondrosarcoma of bone: an assessment of outcome. J Bone Joint Surg Am. 81(3):326-38, 1999
                                25. Cawte TG et al: Chondrosarcoma of the short tubular bones of the hands and feet. Skeletal Radiol. 27(11):625-32, 1998
                                26. Murphey MD et al: Enchondroma versus chondrosarcoma in the appendicular skeleton: differentiating features. Radiographics. 18(5):1213-37; quiz 1244-5, 1998
                                Related Anatomy
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                                Related Differential Diagnoses
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                                References
                                Tables

                                Tables

                                KEY FACTS

                                • Terminology

                                  • Imaging

                                    TERMINOLOGY

                                    • Synonyms

                                      • Primary chondrosarcoma (CS), conventional CS, secondary CS, low-grade peripheral CS
                                    • Definitions

                                      • 4 subtypes defined by location and grade
                                      • Location
                                        • Central: within medullary cavity
                                        • Secondary peripheral: arising within cartilage cap of osteochondroma (OC)
                                          • Lesions arising from enchondroma not considered secondary under WHO classification
                                      • Grade
                                        • Atypical cartilaginous tumor (ACT)/CS, grade 1 (CS1): locally aggressive, hyaline cartilage-producing neoplasm
                                          • ACT: lesions in appendicular skeleton (long and short tubular bones)
                                          • CS1: lesions in axial skeleton (flat bones: pelvis, scapula, skull base)
                                        • CS2 (intermediate grade) and CS3 (high grade): malignant cartilage matrix-producing neoplasms
                                          • Grouped together

                                    IMAGING

                                    • General Features

                                      • Image-Guided Biopsy

                                        • Imaging Recommendations

                                          DIFFERENTIAL DIAGNOSIS

                                            PATHOLOGY

                                            • General Features

                                              • Staging, Grading, & Classification

                                                • Gross Pathologic & Surgical Features

                                                  • Microscopic Features

                                                    CLINICAL ISSUES

                                                    • Presentation

                                                      • Demographics

                                                        • Natural History & Prognosis

                                                          • Treatment

                                                            DIAGNOSTIC CHECKLIST

                                                            • Consider

                                                              Selected References

                                                              1. Lee S et al: Assessment of central cartilaginous tumor of the appendicular bone: inter-observer and intermodality agreement and comparison of diagnostic performance of CT and MRI. Acta Radiol. 63(3):376-86, 2022
                                                              2. Haygood TM et al: Chondroid tumors of bone. Semin Ultrasound CT MR. 42(2):123-33, 2021
                                                              3. Bovée JVMG et al: Central atypical cartilaginous tumour/chondrosarcoma, grade 1. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 370-2, 2020
                                                              4. Bovée JVMG et al: Central chondrosarcoma, grades 2 and 3. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 375-8, 2020
                                                              5. Bovée JVMG et al: Secondary peripheral atypical cartilaginous tumour/chondrosarcoma, grade 1. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 373-4, 2020
                                                              6. Bovée JVMG et al: Secondary peripheral chondrosarcoma, grades 2 and 3. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 379-80, 2020
                                                              7. Davies AM et al: A retrospective validation of an imaging protocol for the management of solitary central cartilage tumours of the proximal humerus and around the knee. Clin Radiol. 74(12):962-71, 2019
                                                              8. Subhawong TK et al: F-18 FDG PET differentiation of benign from malignant chondroid neoplasms: a systematic review of the literature. Skeletal Radiol. 46(9):1233-9, 2017
                                                              9. Ferrer-Santacreu EM et al: Enchondroma versus chondrosarcoma in long bones of appendicular skeleton: clinical and radiological criteria-A follow-Up. J Oncol. 2016:8262079, 2016
                                                              10. Brown MT et al: How safe is curettage of low-grade cartilaginous neoplasms diagnosed by imaging with or without pre-operative needle biopsy? Bone Joint J. 96-B(8):1098-105, 2014
                                                              11. Douis H et al: MRI differentiation of low-grade from high-grade appendicular chondrosarcoma. Eur Radiol. 24(1):232-40, 2014
                                                              12. van Maldegem AM et al: Comprehensive analysis of published studies involving systemic treatment for chondrosarcoma of bone between 2000 and 2013. Clin Sarcoma Res. 4:11, 2014
                                                              13. van Maldegem AM et al: Outcome of advanced, unresectable conventional central chondrosarcoma. Cancer. 120(20):3159-64, 2014
                                                              14. De Coninck T et al: Dynamic contrast-enhanced MR imaging for differentiation between enchondroma and chondrosarcoma. Eur Radiol. 23(11):3140-52, 2013
                                                              15. Italiano A et al: Advanced chondrosarcomas: role of chemotherapy and survival. Ann Oncol. 24(11):2916-22, 2013
                                                              16. Meftah M et al: Long-term results of intralesional curettage and cryosurgery for treatment of low-grade chondrosarcoma. J Bone Joint Surg Am. 95(15):1358-64, 2013
                                                              17. Bui KL et al: Cortical scalloping and cortical penetration by small eccentric chondroid lesions in the long tubular bones: not a sign of malignancy? Skeletal Radiol. 38(8):791-6, 2009
                                                              18. Giuffrida AY et al: Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database. J Bone Joint Surg Am. 91(5):1063-72, 2009
                                                              19. Hallor KH et al: Genomic profiling of chondrosarcoma: chromosomal patterns in central and peripheral tumors. Clin Cancer Res. 15(8):2685-94, 2009
                                                              20. Puri A et al: Chondrosarcoma of bone: does the size of the tumor, the presence of a pathologic fracture, or prior intervention have an impact on local control and survival? J Cancer Res Ther. 5(1):14-9, 2009
                                                              21. Murphey MD et al: From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation. Radiographics. 23(5):1245-78, 2003
                                                              22. Ollivier L et al: Imaging of chondrosarcomas. Cancer Imaging. 4(1):36-8, 2003
                                                              23. Geirnaerdt MJ et al: Cartilaginous tumors: fast contrast-enhanced MR imaging. Radiology. 214(2):539-46, 2000
                                                              24. Lee FY et al: Chondrosarcoma of bone: an assessment of outcome. J Bone Joint Surg Am. 81(3):326-38, 1999
                                                              25. Cawte TG et al: Chondrosarcoma of the short tubular bones of the hands and feet. Skeletal Radiol. 27(11):625-32, 1998
                                                              26. Murphey MD et al: Enchondroma versus chondrosarcoma in the appendicular skeleton: differentiating features. Radiographics. 18(5):1213-37; quiz 1244-5, 1998