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Chordoma
Behrang Amini, MD, PhD; B. J. Manaster, MD, PhD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Clinical Issues

        TERMINOLOGY

        • Synonyms

          • Conventional chordoma, chondroid chordoma, dedifferentiated chordoma, poorly differentiated chordoma
        • Definitions

          • Malignant tumor that derives from notochord
          • Chondroid chordoma: subtype of conventional chordoma with large areas of matrix that mimics hyaline cartilaginous tumors
          • Dedifferentiated chordoma: biphasic appearance with conventional chordoma and high-grade sarcoma
          • Poorly differentiated chordoma: poorly differentiated neoplasm with notochordal differentiation

        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. Akinduro OO et al: Targeted therapy for chordoma: key molecular signaling pathways and the role of multimodal therapy. Target Oncol. 16(3):325-37, 2021
                                        2. Tirabosco R et al: Benign notochordal cell tumour. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 449-50, 2020
                                        3. Tirabosco R et al: Conventional chordoma. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 451-3, 2020
                                        4. Zuckerman SL et al: Predictive value of preoperative magnetic resonance imaging findings for survival and local recurrence in patients undergoing en bloc resection of sacral chordomas. Neurosurgery. 85(6):834-42, 2019
                                        5. Stacchiotti S et al: Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol. 28(6):1230-42, 2017
                                        6. Lebellec L et al: Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses. Crit Rev Oncol Hematol. 95(1):125-31, 2015
                                        7. 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. 17(5):725-32, 2015
                                        8. Stacchiotti S et al: Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol. 16(2):e71-83, 2015
                                        9. Varga PP et al: Surgical treatment of sacral chordoma: prognostic variables for local recurrence and overall survival. Eur Spine J. 24(5):1092-101, 2015
                                        10. Kayani B et al: A review of the surgical management of sacral chordoma. Eur J Surg Oncol. 40(11):1412-20, 2014
                                        11. Kayani B et al: Prognostic factors in the operative management of dedifferentiated sacral chordomas. Neurosurgery. 75(3):269-75; discussion 275, 2014
                                        12. Orguc S et al: Primary tumors of the spine. Semin Musculoskelet Radiol. 18(3):280-99, 2014
                                        13. Wang T et al: Role of apparent diffusion coefficients with diffusion-weighted magnetic resonance imaging in differentiating between benign and malignant bone tumors. World J Surg Oncol. 12:365, 2014
                                        14. Bernard SA et al: Primary osseous tumors of the spine. Semin Musculoskelet Radiol. 17(2):203-20, 2013
                                        15. 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
                                        16. 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
                                        17. Farsad K et al: Sacral chordoma. Radiographics. 29(5):1525-30, 2009
                                        18. Martin MP et al: Intradural drop metastasis of a clival chordoma. J Clin Neurosci. 16(8):1105-7, 2009
                                        19. Presneau N et al: Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway. Br J Cancer. 100(9):1406-14, 2009
                                        20. 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
                                        21. Gerber S et al: Imaging of sacral tumours. Skeletal Radiol. 37(4):277-89, 2008
                                        22. Hanna SA et al: Dedifferentiated chordoma: a report of four cases arising 'de novo'. J Bone Joint Surg Br. 90(5):652-6, 2008
                                        23. Hanna SA et al: Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res. 466(9):2217-23, 2008
                                        24. 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
                                        25. Park SA et al: F-18 FDG PET/CT evaluation of sacrococcygeal chordoma. Clin Nucl Med. 33(12):906-8, 2008
                                        26. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                        27. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                        28. Casali PG et al: Chordoma. Curr Opin Oncol. 19(4):367-70, 2007
                                        29. Soudack M et al: Imaging features of posterior mediastinal chordoma in a child. Pediatr Radiol. 37(5):492-7, 2007
                                        30. Teichgräber V et al: Percutaneous MR-guided radiofrequency ablation of recurrent sacrococcygeal chordomas. AJR Am J Roentgenol. 187(2):571-4, 2006
                                        31. Anis N et al: Use of radio-frequency ablation for the palliative treatment of sacral chordoma. AJNR Am J Neuroradiol. 25(9):1589-91, 2004
                                        32. Neeman Z et al: Percutaneous radiofrequency ablation of chordoma. AJR Am J Roentgenol. 179(5):1330-2, 2002
                                        33. Resnick D: Diagnosis of Bone and Joint Disorders. 4th ed. 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

                                                • Conventional chordoma, chondroid chordoma, dedifferentiated chordoma, poorly differentiated chordoma
                                              • Definitions

                                                • Malignant tumor that derives from notochord
                                                • Chondroid chordoma: subtype of conventional chordoma with large areas of matrix that mimics hyaline cartilaginous tumors
                                                • Dedifferentiated chordoma: biphasic appearance with conventional chordoma and high-grade sarcoma
                                                • Poorly differentiated chordoma: poorly differentiated neoplasm with notochordal differentiation

                                              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. Akinduro OO et al: Targeted therapy for chordoma: key molecular signaling pathways and the role of multimodal therapy. Target Oncol. 16(3):325-37, 2021
                                                                              2. Tirabosco R et al: Benign notochordal cell tumour. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 449-50, 2020
                                                                              3. Tirabosco R et al: Conventional chordoma. In Fletcher CDM et al: WHO Classification of Tumours: Tumours of Soft Tissue and Bone. IARC Press. 451-3, 2020
                                                                              4. Zuckerman SL et al: Predictive value of preoperative magnetic resonance imaging findings for survival and local recurrence in patients undergoing en bloc resection of sacral chordomas. Neurosurgery. 85(6):834-42, 2019
                                                                              5. Stacchiotti S et al: Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol. 28(6):1230-42, 2017
                                                                              6. Lebellec L et al: Molecular targeted therapies in advanced or metastatic chordoma patients: facts and hypotheses. Crit Rev Oncol Hematol. 95(1):125-31, 2015
                                                                              7. 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. 17(5):725-32, 2015
                                                                              8. Stacchiotti S et al: Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol. 16(2):e71-83, 2015
                                                                              9. Varga PP et al: Surgical treatment of sacral chordoma: prognostic variables for local recurrence and overall survival. Eur Spine J. 24(5):1092-101, 2015
                                                                              10. Kayani B et al: A review of the surgical management of sacral chordoma. Eur J Surg Oncol. 40(11):1412-20, 2014
                                                                              11. Kayani B et al: Prognostic factors in the operative management of dedifferentiated sacral chordomas. Neurosurgery. 75(3):269-75; discussion 275, 2014
                                                                              12. Orguc S et al: Primary tumors of the spine. Semin Musculoskelet Radiol. 18(3):280-99, 2014
                                                                              13. Wang T et al: Role of apparent diffusion coefficients with diffusion-weighted magnetic resonance imaging in differentiating between benign and malignant bone tumors. World J Surg Oncol. 12:365, 2014
                                                                              14. Bernard SA et al: Primary osseous tumors of the spine. Semin Musculoskelet Radiol. 17(2):203-20, 2013
                                                                              15. 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
                                                                              16. 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
                                                                              17. Farsad K et al: Sacral chordoma. Radiographics. 29(5):1525-30, 2009
                                                                              18. Martin MP et al: Intradural drop metastasis of a clival chordoma. J Clin Neurosci. 16(8):1105-7, 2009
                                                                              19. Presneau N et al: Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway. Br J Cancer. 100(9):1406-14, 2009
                                                                              20. 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
                                                                              21. Gerber S et al: Imaging of sacral tumours. Skeletal Radiol. 37(4):277-89, 2008
                                                                              22. Hanna SA et al: Dedifferentiated chordoma: a report of four cases arising 'de novo'. J Bone Joint Surg Br. 90(5):652-6, 2008
                                                                              23. Hanna SA et al: Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res. 466(9):2217-23, 2008
                                                                              24. 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
                                                                              25. Park SA et al: F-18 FDG PET/CT evaluation of sacrococcygeal chordoma. Clin Nucl Med. 33(12):906-8, 2008
                                                                              26. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                                                              27. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                                                              28. Casali PG et al: Chordoma. Curr Opin Oncol. 19(4):367-70, 2007
                                                                              29. Soudack M et al: Imaging features of posterior mediastinal chordoma in a child. Pediatr Radiol. 37(5):492-7, 2007
                                                                              30. Teichgräber V et al: Percutaneous MR-guided radiofrequency ablation of recurrent sacrococcygeal chordomas. AJR Am J Roentgenol. 187(2):571-4, 2006
                                                                              31. Anis N et al: Use of radio-frequency ablation for the palliative treatment of sacral chordoma. AJNR Am J Neuroradiol. 25(9):1589-91, 2004
                                                                              32. Neeman Z et al: Percutaneous radiofrequency ablation of chordoma. AJR Am J Roentgenol. 179(5):1330-2, 2002
                                                                              33. Resnick D: Diagnosis of Bone and Joint Disorders. 4th ed. WB Saunders. 4014-23, 2002