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Chordoma
Cheryl A. Petersilge, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Malignant tumor arising from notochord remnants

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Nonvascular Interventions

                        • Nuclear Medicine Findings

                          • Imaging Recommendations

                            • Angiographic Findings

                              DIFFERENTIAL DIAGNOSIS

                                PATHOLOGY

                                • General Features

                                  • Gross Pathologic & Surgical Features

                                    • Microscopic Features

                                      CLINICAL ISSUES

                                      • Presentation

                                        • Demographics

                                          • Natural History & Prognosis

                                            • Treatment

                                              DIAGNOSTIC CHECKLIST

                                              • Consider

                                                • Image Interpretation Pearls

                                                  Selected References

                                                  1. Osaka S et al: Long-term outcome following surgical treatment of sacral chordoma. J Surg Oncol. 109(3):184-8, 2014
                                                  2. Bernard SA et al: Primary osseous tumors of the spine. Semin Musculoskelet Radiol. 17(2):203-20, 2013
                                                  3. Iorgulescu JB et al: Benign notochordal cell tumors of the spine: natural history of 8 patients with histologically confirmed lesions. Neurosurgery. 73(3):411-6, 2013
                                                  4. Yamada Y et al: Preliminary results of high-dose single-fraction radiotherapy for the management of chordomas of the spine and sacrum. Neurosurgery. 73(4):673-80; discussion 680, 2013
                                                  5. Wang Y et al: Primary chordomas of the cervical spine: a consecutive series of 14 surgically managed cases. J Neurosurg Spine. 17(4):292-9, 2012
                                                  6. Al-Adra D et al: Pediatric metastatic sacrococcygeal chordoma treated with surgery. Eur J Pediatr Surg. 21(3):196-8, 2011
                                                  7. McMaster ML et al: Clinical features distinguish childhood chordoma associated with tuberous sclerosis complex (TSC) from chordoma in the general paediatric population. J Med Genet. 48(7):444-9, 2011
                                                  8. Choi GH et al: Pediatric cervical chordoma: report of two cases and a review of the current literature. Childs Nerv Syst. 26(6):835-40, 2010
                                                  9. Brennan PM et al: Chordoma masquerading as a nerve root tumour -- a clinical lesson. Br J Radiol. 82(983):e231-4, 2009
                                                  10. Menezes AH: Craniovertebral junction neoplasms in the pediatric population. Childs Nerv Syst. 24(10):1173-86, 2008
                                                  11. McMaster ML et al: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 12(1):1-11, 2001
                                                  12. Tai PT et al: Management issues in chordoma: a case series. Clin Oncol (R Coll Radiol). 12(2):80-6, 2000
                                                  13. Gelabert-Gonzalez M et al: Intradural cervical chordoma. Case report. J Neurosurg Sci. 43(2):159-62, 1999
                                                  14. York JE et al: Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 44(1):74-9; discussion 79-80, 1999
                                                  15. Logroscino CA et al: Chordoma: long-term evaluation of 15 cases treated surgically. Chir Organi Mov. 83(1-2):87-103, 1998
                                                  16. Murphy JM et al: CT and MRI appearances of a thoracic chordoma. Eur Radiol. 8(9):1677-9, 1998
                                                  17. Boriani S et al: Primary bone tumors of the spine. Terminology and surgical staging. Spine. 22(9):1036-44, 1997
                                                  18. Boriani S et al: Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. 21(13):1569-77, 1996
                                                  19. Catton C et al: Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol. 41(1):67-72, 1996
                                                  20. Klekamp J et al: Spinal chordomas--results of treatment over a 17-year period. Acta Neurochir (Wien). 138(5):514-9, 1996
                                                  21. Murphey MD et al: From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics. 16(5):1131-58, 1996
                                                  22. Fagundes MA et al: Radiation therapy for chordomas of the base of skull and cervical spine: patterns of failure and outcome after relapse. Int J Radiat Oncol Biol Phys. 33(3):579-84, 1995
                                                  23. Wojno KJ et al: Chondroid chordomas and low-grade chondrosarcomas of the craniospinal axis. An immunohistochemical analysis of 17 cases. Am J Surg Pathol. 16(12):1144-52, 1992
                                                  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

                                                            • Diagnostic Checklist

                                                              TERMINOLOGY

                                                              • Definitions

                                                                • Malignant tumor arising from notochord remnants

                                                              IMAGING

                                                              • General Features

                                                                • Radiographic Findings

                                                                  • CT Findings

                                                                    • MR Findings

                                                                      • Nonvascular Interventions

                                                                        • Nuclear Medicine Findings

                                                                          • Imaging Recommendations

                                                                            • Angiographic Findings

                                                                              DIFFERENTIAL DIAGNOSIS

                                                                                PATHOLOGY

                                                                                • General Features

                                                                                  • Gross Pathologic & Surgical Features

                                                                                    • Microscopic Features

                                                                                      CLINICAL ISSUES

                                                                                      • Presentation

                                                                                        • Demographics

                                                                                          • Natural History & Prognosis

                                                                                            • Treatment

                                                                                              DIAGNOSTIC CHECKLIST

                                                                                              • Consider

                                                                                                • Image Interpretation Pearls

                                                                                                  Selected References

                                                                                                  1. Osaka S et al: Long-term outcome following surgical treatment of sacral chordoma. J Surg Oncol. 109(3):184-8, 2014
                                                                                                  2. Bernard SA et al: Primary osseous tumors of the spine. Semin Musculoskelet Radiol. 17(2):203-20, 2013
                                                                                                  3. Iorgulescu JB et al: Benign notochordal cell tumors of the spine: natural history of 8 patients with histologically confirmed lesions. Neurosurgery. 73(3):411-6, 2013
                                                                                                  4. Yamada Y et al: Preliminary results of high-dose single-fraction radiotherapy for the management of chordomas of the spine and sacrum. Neurosurgery. 73(4):673-80; discussion 680, 2013
                                                                                                  5. Wang Y et al: Primary chordomas of the cervical spine: a consecutive series of 14 surgically managed cases. J Neurosurg Spine. 17(4):292-9, 2012
                                                                                                  6. Al-Adra D et al: Pediatric metastatic sacrococcygeal chordoma treated with surgery. Eur J Pediatr Surg. 21(3):196-8, 2011
                                                                                                  7. McMaster ML et al: Clinical features distinguish childhood chordoma associated with tuberous sclerosis complex (TSC) from chordoma in the general paediatric population. J Med Genet. 48(7):444-9, 2011
                                                                                                  8. Choi GH et al: Pediatric cervical chordoma: report of two cases and a review of the current literature. Childs Nerv Syst. 26(6):835-40, 2010
                                                                                                  9. Brennan PM et al: Chordoma masquerading as a nerve root tumour -- a clinical lesson. Br J Radiol. 82(983):e231-4, 2009
                                                                                                  10. Menezes AH: Craniovertebral junction neoplasms in the pediatric population. Childs Nerv Syst. 24(10):1173-86, 2008
                                                                                                  11. McMaster ML et al: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 12(1):1-11, 2001
                                                                                                  12. Tai PT et al: Management issues in chordoma: a case series. Clin Oncol (R Coll Radiol). 12(2):80-6, 2000
                                                                                                  13. Gelabert-Gonzalez M et al: Intradural cervical chordoma. Case report. J Neurosurg Sci. 43(2):159-62, 1999
                                                                                                  14. York JE et al: Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 44(1):74-9; discussion 79-80, 1999
                                                                                                  15. Logroscino CA et al: Chordoma: long-term evaluation of 15 cases treated surgically. Chir Organi Mov. 83(1-2):87-103, 1998
                                                                                                  16. Murphy JM et al: CT and MRI appearances of a thoracic chordoma. Eur Radiol. 8(9):1677-9, 1998
                                                                                                  17. Boriani S et al: Primary bone tumors of the spine. Terminology and surgical staging. Spine. 22(9):1036-44, 1997
                                                                                                  18. Boriani S et al: Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. 21(13):1569-77, 1996
                                                                                                  19. Catton C et al: Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol. 41(1):67-72, 1996
                                                                                                  20. Klekamp J et al: Spinal chordomas--results of treatment over a 17-year period. Acta Neurochir (Wien). 138(5):514-9, 1996
                                                                                                  21. Murphey MD et al: From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics. 16(5):1131-58, 1996
                                                                                                  22. Fagundes MA et al: Radiation therapy for chordomas of the base of skull and cervical spine: patterns of failure and outcome after relapse. Int J Radiat Oncol Biol Phys. 33(3):579-84, 1995
                                                                                                  23. Wojno KJ et al: Chondroid chordomas and low-grade chondrosarcomas of the craniospinal axis. An immunohistochemical analysis of 17 cases. Am J Surg Pathol. 16(12):1144-52, 1992