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Chordoma, Craniovertebral Junction
Cheryl A. Petersilge, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Definitions

            • Malignant tumor arising from notochord remnants
            • Most common primary malignancy of spine

          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. 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
                                              2. Brennan PM et al: Chordoma masquerading as a nerve root tumour -- a clinical lesson. Br J Radiol. 82(983):e231-4, 2009
                                              3. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                              4. Chugh R et al: Chordoma: the nonsarcoma primary bone tumor. Oncologist. 12(11):1344-50, 2007
                                              5. Boriani S et al: Chordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976). 31(4):493-503, 2006
                                              6. Takeyama J et al: Notochordal remnant-derived mass: ecchordosis physaliphora or chordoma? Pathology. 38(6):599-600, 2006
                                              7. Maclean FM et al: Chordoma: radiological-pathological correlation. Australas Radiol. 49(4):261-8, 2005
                                              8. Shinde SV et al: Craniospinal dissemination of clival chondroid chordoma. J Postgrad Med. 51(3):220-2, 2005
                                              9. Baratti D et al: Chordoma: natural history and results in 28 patients treated at a single institution. Ann Surg Oncol. 10(3):291-6, 2003
                                              10. Erdem E et al: Comprehensive review of intracranial chordoma. Radiographics. 23(4):995-1009, 2003
                                              11. Noel G et al: Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure. Strahlenther Onkol. 179(4):241-8, 2003
                                              12. Smolders D et al: Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol. 32(6):343-50, 2003
                                              13. Carpentier A et al: Suboccipital and cervical chordomas: the value of aggressive treatment at first presentation of the disease. J Neurosurg. 97(5):1070-7, 2002
                                              14. Leone A et al: Chordoma of the low cervical spine presenting with Horner's syndrome. Eur Radiol. 12 Suppl 3:S43-7, 2002
                                              15. Steenberghs J et al: Intradural chordoma without bone involvement. Case report and review of the literature. J Neurosurg. 97(1 Suppl):94-7, 2002
                                              16. Arnautovic KI et al: Surgical seeding of chordomas. J Neurosurg. 95(5):798-803, 2001
                                              17. Crapanzano JP et al: Chordoma: a cytologic study with histologic and radiologic correlation. Cancer. 93(1):40-51, 2001
                                              18. McMaster ML et al: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 12(1):1-11, 2001
                                              19. Scheil S et al: Genome-wide analysis of sixteen chordomas by comparative genomic hybridization and cytogenetics of the first human chordoma cell line, U-CH1. Genes Chromosomes Cancer. 32(3):203-11, 2001
                                              20. Soo MY: Chordoma: review of clinicoradiological features and factors affecting survival. Australas Radiol. 45(4):427-34, 2001
                                              21. Bergh P et al: Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 88(9):2122-34, 2000
                                              22. Tai PT et al: Management issues in chordoma: a case series. Clin Oncol (R Coll Radiol). 12(2):80-6, 2000
                                              23. Gelabert-Gonzalez M et al: Intradural cervical chordoma. Case report. J Neurosurg Sci. 43(2):159-62, 1999
                                              24. Wippold FJ 2nd et al: Clinical and imaging features of cervical chordoma. AJR Am J Roentgenol. 172(5):1423-6, 1999
                                              25. York JE et al: Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 44(1):74-9; discussion 79-80, 1999
                                              26. Logroscino CA et al: Chordoma: long-term evaluation of 15 cases treated surgically. Chir Organi Mov. 83(1-2):87-103, 1998
                                              27. Murphy JM et al: CT and MRI appearances of a thoracic chordoma. Eur Radiol. 8(9):1677-9, 1998
                                              28. Ng SH et al: Cervical ecchordosis physaliphora: CT and MR features. Br J Radiol. 71(843):329-31, 1998
                                              29. Boriani S et al: Primary bone tumors of the spine. Terminology and surgical staging. Spine. 22(9):1036-44, 1997
                                              30. Boriani S et al: Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. 21(13):1569-77, 1996
                                              31. Catton C et al: Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol. 41(1):67-72, 1996
                                              32. Klekamp J et al: Spinal chordomas--results of treatment over a 17-year period. Acta Neurochir (Wien). 138(5):514-9, 1996
                                              33. Murphey MD et al: From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics. 16(5):1131-58, 1996
                                              34. 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
                                              35. 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
                                              36. Firooznia H et al: Chordoma: radiologic evaluation of 20 cases. AJR Am J Roentgenol. 127(5):797-805, 1976
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Clinical Issues

                                                      TERMINOLOGY

                                                      • Definitions

                                                        • Malignant tumor arising from notochord remnants
                                                        • Most common primary malignancy of spine

                                                      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. 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
                                                                                          2. Brennan PM et al: Chordoma masquerading as a nerve root tumour -- a clinical lesson. Br J Radiol. 82(983):e231-4, 2009
                                                                                          3. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                                                                          4. Chugh R et al: Chordoma: the nonsarcoma primary bone tumor. Oncologist. 12(11):1344-50, 2007
                                                                                          5. Boriani S et al: Chordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976). 31(4):493-503, 2006
                                                                                          6. Takeyama J et al: Notochordal remnant-derived mass: ecchordosis physaliphora or chordoma? Pathology. 38(6):599-600, 2006
                                                                                          7. Maclean FM et al: Chordoma: radiological-pathological correlation. Australas Radiol. 49(4):261-8, 2005
                                                                                          8. Shinde SV et al: Craniospinal dissemination of clival chondroid chordoma. J Postgrad Med. 51(3):220-2, 2005
                                                                                          9. Baratti D et al: Chordoma: natural history and results in 28 patients treated at a single institution. Ann Surg Oncol. 10(3):291-6, 2003
                                                                                          10. Erdem E et al: Comprehensive review of intracranial chordoma. Radiographics. 23(4):995-1009, 2003
                                                                                          11. Noel G et al: Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure. Strahlenther Onkol. 179(4):241-8, 2003
                                                                                          12. Smolders D et al: Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol. 32(6):343-50, 2003
                                                                                          13. Carpentier A et al: Suboccipital and cervical chordomas: the value of aggressive treatment at first presentation of the disease. J Neurosurg. 97(5):1070-7, 2002
                                                                                          14. Leone A et al: Chordoma of the low cervical spine presenting with Horner's syndrome. Eur Radiol. 12 Suppl 3:S43-7, 2002
                                                                                          15. Steenberghs J et al: Intradural chordoma without bone involvement. Case report and review of the literature. J Neurosurg. 97(1 Suppl):94-7, 2002
                                                                                          16. Arnautovic KI et al: Surgical seeding of chordomas. J Neurosurg. 95(5):798-803, 2001
                                                                                          17. Crapanzano JP et al: Chordoma: a cytologic study with histologic and radiologic correlation. Cancer. 93(1):40-51, 2001
                                                                                          18. McMaster ML et al: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 12(1):1-11, 2001
                                                                                          19. Scheil S et al: Genome-wide analysis of sixteen chordomas by comparative genomic hybridization and cytogenetics of the first human chordoma cell line, U-CH1. Genes Chromosomes Cancer. 32(3):203-11, 2001
                                                                                          20. Soo MY: Chordoma: review of clinicoradiological features and factors affecting survival. Australas Radiol. 45(4):427-34, 2001
                                                                                          21. Bergh P et al: Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 88(9):2122-34, 2000
                                                                                          22. Tai PT et al: Management issues in chordoma: a case series. Clin Oncol (R Coll Radiol). 12(2):80-6, 2000
                                                                                          23. Gelabert-Gonzalez M et al: Intradural cervical chordoma. Case report. J Neurosurg Sci. 43(2):159-62, 1999
                                                                                          24. Wippold FJ 2nd et al: Clinical and imaging features of cervical chordoma. AJR Am J Roentgenol. 172(5):1423-6, 1999
                                                                                          25. York JE et al: Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 44(1):74-9; discussion 79-80, 1999
                                                                                          26. Logroscino CA et al: Chordoma: long-term evaluation of 15 cases treated surgically. Chir Organi Mov. 83(1-2):87-103, 1998
                                                                                          27. Murphy JM et al: CT and MRI appearances of a thoracic chordoma. Eur Radiol. 8(9):1677-9, 1998
                                                                                          28. Ng SH et al: Cervical ecchordosis physaliphora: CT and MR features. Br J Radiol. 71(843):329-31, 1998
                                                                                          29. Boriani S et al: Primary bone tumors of the spine. Terminology and surgical staging. Spine. 22(9):1036-44, 1997
                                                                                          30. Boriani S et al: Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. 21(13):1569-77, 1996
                                                                                          31. Catton C et al: Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol. 41(1):67-72, 1996
                                                                                          32. Klekamp J et al: Spinal chordomas--results of treatment over a 17-year period. Acta Neurochir (Wien). 138(5):514-9, 1996
                                                                                          33. Murphey MD et al: From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics. 16(5):1131-58, 1996
                                                                                          34. 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
                                                                                          35. 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
                                                                                          36. Firooznia H et al: Chordoma: radiologic evaluation of 20 cases. AJR Am J Roentgenol. 127(5):797-805, 1976