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Chordoma
B.J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Chondroid chordoma, dedifferentiated chordoma
        • Definitions

          • Low- to intermediate-grade malignant tumor that recapitulates notochord

        IMAGING

        • General Features

          • Imaging Recommendations

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    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. Lebellec L et al: Molecular targeted therapies in advanced or metastatic chordoma patients: Facts and hypotheses. Crit Rev Oncol Hematol. ePub, 2015
                                        2. Kayani B et al: A review of the surgical management of sacral chordoma. Eur J Surg Oncol. 40(11):1412-20, 2014
                                        3. Kayani B et al: Prognostic factors in the operative management of dedifferentiated sacral chordomas. Neurosurgery. 75(3):269-75; discussion 275, 2014
                                        4. Meng T et al: Clinical features and prognostic factors of patients with chordoma in the spine: a retrospective analysis of 153 patients in a single center. Neuro Oncol. ePub, 2014
                                        5. Orguc S et al: Primary tumors of the spine. Semin Musculoskelet Radiol. 18(3):280-99, 2014
                                        6. Varga PP et al: Surgical treatment of sacral chordoma: prognostic variables for local recurrence and overall survival. Eur Spine J. ePub, 2014
                                        7. Si MJ et al: Differentiation of primary chordoma, giant cell tumor and schwannoma of the sacrum by CT and MRI. Eur J Radiol. 82(12):2309-15, 2013
                                        8. Chou WC et al: De novo dedifferentiated chordoma of the sacrum: a case report and review of the literature. Chang Gung Med J. 32(3):330-5, 2009
                                        9. Farsad K et al: Sacral chordoma. Radiographics. 29(5):1525-30, 2009
                                        10. Martin MP et al: Intradural drop metastasis of a clival chordoma. J Clin Neurosci. 16(8):1105-7, 2009
                                        11. Presneau N et al: Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway. Br J Cancer. 100(9):1406-14, 2009
                                        12. Torres MA et al: Optimal treatment planning for skull base chordoma: photons, protons, or a combination of both? Int J Radiat Oncol Biol Phys. 74(4):1033-9, 2009
                                        13. Gerber S et al: Imaging of sacral tumours. Skeletal Radiol. 37(4):277-89, 2008
                                        14. Hanna SA et al: Dedifferentiated chordoma: a report of four cases arising 'de novo'. J Bone Joint Surg Br. 90(5):652-6, 2008
                                        15. Hanna SA et al: Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res. 466(9):2217-23, 2008
                                        16. Nguyen QN et al: Emerging role of proton beam radiation therapy for chordoma and chondrosarcoma of the skull base. Curr Oncol Rep. 10(4):338-43, 2008
                                        17. Park SA et al: F-18 FDG PET/CT evaluation of sacrococcygeal chordoma. Clin Nucl Med. 33(12):906-8, 2008
                                        18. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                        19. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                        20. Casali PG et al: Chordoma. Curr Opin Oncol. 19(4):367-70, 2007
                                        21. Soudack M et al: Imaging features of posterior mediastinal chordoma in a child. Pediatr Radiol. 37(5):492-7, 2007
                                        22. Teichgräber V et al: Percutaneous MR-guided radiofrequency ablation of recurrent sacrococcygeal chordomas. AJR Am J Roentgenol. 187(2):571-4, 2006
                                        23. Anis N et al. Use of radiofrequency ablation for the palliative treatment of sacral chordoma. Am J Neuroradiol. 25: 1589-91, 2004
                                        24. Mirra JM et al: Chordoma. In Fletcher CDM et al: World Health Organization Classification of Tumors: Tumors of Soft Tissue and Bone. Lyon: IARC Press. 316-17, 2002
                                        25. Neeman Z et al: Percutaneous radiofrequency ablation of chordoma. AJR Am J Roentgenol. 179(5):1330-2, 2002
                                        26. Resnick D: Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: WB Saunders. 4014-23, 2002
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Synonyms

                                                • Chondroid chordoma, dedifferentiated chordoma
                                              • Definitions

                                                • Low- to intermediate-grade malignant tumor that recapitulates notochord

                                              IMAGING

                                              • General Features

                                                • Imaging Recommendations

                                                  • Radiographic Findings

                                                    • CT Findings

                                                      • MR Findings

                                                        • Nuclear Medicine Findings

                                                          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. Lebellec L et al: Molecular targeted therapies in advanced or metastatic chordoma patients: Facts and hypotheses. Crit Rev Oncol Hematol. ePub, 2015
                                                                              2. Kayani B et al: A review of the surgical management of sacral chordoma. Eur J Surg Oncol. 40(11):1412-20, 2014
                                                                              3. Kayani B et al: Prognostic factors in the operative management of dedifferentiated sacral chordomas. Neurosurgery. 75(3):269-75; discussion 275, 2014
                                                                              4. Meng T et al: Clinical features and prognostic factors of patients with chordoma in the spine: a retrospective analysis of 153 patients in a single center. Neuro Oncol. ePub, 2014
                                                                              5. Orguc S et al: Primary tumors of the spine. Semin Musculoskelet Radiol. 18(3):280-99, 2014
                                                                              6. Varga PP et al: Surgical treatment of sacral chordoma: prognostic variables for local recurrence and overall survival. Eur Spine J. ePub, 2014
                                                                              7. Si MJ et al: Differentiation of primary chordoma, giant cell tumor and schwannoma of the sacrum by CT and MRI. Eur J Radiol. 82(12):2309-15, 2013
                                                                              8. Chou WC et al: De novo dedifferentiated chordoma of the sacrum: a case report and review of the literature. Chang Gung Med J. 32(3):330-5, 2009
                                                                              9. Farsad K et al: Sacral chordoma. Radiographics. 29(5):1525-30, 2009
                                                                              10. Martin MP et al: Intradural drop metastasis of a clival chordoma. J Clin Neurosci. 16(8):1105-7, 2009
                                                                              11. Presneau N et al: Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway. Br J Cancer. 100(9):1406-14, 2009
                                                                              12. Torres MA et al: Optimal treatment planning for skull base chordoma: photons, protons, or a combination of both? Int J Radiat Oncol Biol Phys. 74(4):1033-9, 2009
                                                                              13. Gerber S et al: Imaging of sacral tumours. Skeletal Radiol. 37(4):277-89, 2008
                                                                              14. Hanna SA et al: Dedifferentiated chordoma: a report of four cases arising 'de novo'. J Bone Joint Surg Br. 90(5):652-6, 2008
                                                                              15. Hanna SA et al: Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res. 466(9):2217-23, 2008
                                                                              16. Nguyen QN et al: Emerging role of proton beam radiation therapy for chordoma and chondrosarcoma of the skull base. Curr Oncol Rep. 10(4):338-43, 2008
                                                                              17. Park SA et al: F-18 FDG PET/CT evaluation of sacrococcygeal chordoma. Clin Nucl Med. 33(12):906-8, 2008
                                                                              18. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                                                              19. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                                                              20. Casali PG et al: Chordoma. Curr Opin Oncol. 19(4):367-70, 2007
                                                                              21. Soudack M et al: Imaging features of posterior mediastinal chordoma in a child. Pediatr Radiol. 37(5):492-7, 2007
                                                                              22. Teichgräber V et al: Percutaneous MR-guided radiofrequency ablation of recurrent sacrococcygeal chordomas. AJR Am J Roentgenol. 187(2):571-4, 2006
                                                                              23. Anis N et al. Use of radiofrequency ablation for the palliative treatment of sacral chordoma. Am J Neuroradiol. 25: 1589-91, 2004
                                                                              24. Mirra JM et al: Chordoma. In Fletcher CDM et al: World Health Organization Classification of Tumors: Tumors of Soft Tissue and Bone. Lyon: IARC Press. 316-17, 2002
                                                                              25. Neeman Z et al: Percutaneous radiofrequency ablation of chordoma. AJR Am J Roentgenol. 179(5):1330-2, 2002
                                                                              26. Resnick D: Diagnosis of Bone and Joint Disorders. 4th ed. Philadelphia: WB Saunders. 4014-23, 2002