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Chordoma
Kevin R. Moore, MD
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17
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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

                            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

                                                • Image Interpretation Pearls

                                                  Selected References

                                                  1. Antonelli M et al: SMARCB1/INI1 involvement in pediatric chordoma: a mutational and immunohistochemical analysis. Am J Surg Pathol. 41(1):56-61, 2017
                                                  2. Ramesh S et al: Multicentric chordoma in a child. J Pediatr Neurosci. 12(2):156-9, 2017
                                                  3. Zhou J et al: Prognostic factors in patients with spinal chordoma: an integrative analysis of 682 patients. Neurosurgery. 81(5):812-23, 2017
                                                  4. Sebro R et al: Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas. BMC Med Imaging. 16(1):53, 2016
                                                  5. Tsitouras V et al: Management and outcome of chordomas in the pediatric population: the Hospital for Sick Children experience and review of the literature. J Clin Neurosci. 34:169-76, 2016
                                                  6. Osaka S et al: Long-term outcome following surgical treatment of sacral chordoma. J Surg Oncol. 109(3):184-8, 2014
                                                  7. 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
                                                  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. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                                  11. Takeyama J et al: Giant chordoma occupying the whole abdominal cavity. Pathology. 40(3):313-4, 2008
                                                  12. Chugh R et al: Chordoma: the nonsarcoma primary bone tumor. Oncologist. 12(11):1344-50, 2007
                                                  13. Boriani S et al: Chordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976). 31(4):493-503, 2006
                                                  14. Takeyama J et al: Notochordal remnant-derived mass: ecchordosis physaliphora or chordoma? Pathology. 38(6):599-600, 2006
                                                  15. Maclean FM et al: Chordoma: radiological-pathological correlation. Australas Radiol. 49(4):261-8, 2005
                                                  16. Shinde SV et al: Craniospinal dissemination of clival chondroid chordoma. J Postgrad Med. 51(3):220-2, 2005
                                                  17. 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
                                                  18. 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
                                                  19. Smolders D et al: Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol. 32(6):343-50, 2003
                                                  20. Bayar MA et al: Spinal chordoma of the terminal filum. Case report. J Neurosurg. 96(2 Suppl):236-8, 2002
                                                  21. 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
                                                  22. Delank KS et al: Metastasizing chordoma of the lumbar spine. Eur Spine J. 11(2):167-71, 2002
                                                  23. Leone A et al: Chordoma of the low cervical spine presenting with Horner's syndrome. Eur Radiol. 12 Suppl 3:S43-7, 2002
                                                  24. Steenberghs J et al: Intradural chordoma without bone involvement. Case report and review of the literature. J Neurosurg. 97(1 Suppl):94-7, 2002
                                                  25. Arnautovic KI et al: Surgical seeding of chordomas. J Neurosurg. 95(5):798-803, 2001
                                                  26. Bosma JJ et al: En bloc removal of the lower lumbar vertebral body for chordoma. Report of two cases. J Neurosurg. 94(2 Suppl):284-91, 2001
                                                  27. Crapanzano JP et al: Chordoma: a cytologic study with histologic and radiologic correlation. Cancer. 93(1):40-51, 2001
                                                  28. McMaster ML et al: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 12(1):1-11, 2001
                                                  29. 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
                                                  30. Soo MY: Chordoma: review of clinicoradiological features and factors affecting survival. Australas Radiol. 45(4):427-34, 2001
                                                  31. 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
                                                  32. Tai PT et al: Management issues in chordoma: a case series. Clin Oncol (R Coll Radiol). 12(2):80-6, 2000
                                                  33. Cheng EY et al: Lumbosacral chordoma. Prognostic factors and treatment. Spine. 24(16):1639-45, 1999
                                                  34. Gelabert-Gonzalez M et al: Intradural cervical chordoma. Case report. J Neurosurg Sci. 43(2):159-62, 1999
                                                  35. Wippold FJ 2nd et al: Clinical and imaging features of cervical chordoma. AJR Am J Roentgenol. 172(5):1423-6, 1999
                                                  36. York JE et al: Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 44(1):74-9; discussion 79-80, 1999
                                                  37. Logroscino CA et al: Chordoma: long-term evaluation of 15 cases treated surgically. Chir Organi Mov. 83(1-2):87-103, 1998
                                                  38. Murphy JM et al: CT and MRI appearances of a thoracic chordoma. Eur Radiol. 8(9):1677-9, 1998
                                                  39. Ng SH et al: Cervical ecchordosis physaliphora: CT and MR features. Br J Radiol. 71(843):329-31, 1998
                                                  40. Boriani S et al: Primary bone tumors of the spine. Terminology and surgical staging. Spine. 22(9):1036-44, 1997
                                                  41. Boriani S et al: Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. 21(13):1569-77, 1996
                                                  42. Catton C et al: Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol. 41(1):67-72, 1996
                                                  43. Klekamp J et al: Spinal chordomas--results of treatment over a 17-year period. Acta Neurochir (Wien). 138(5):514-9, 1996
                                                  44. Murphey MD et al: From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics. 16(5):1131-58, 1996
                                                  45. 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
                                                  46. Hall WA et al: Sacrococcygeal chordoma metastatic to the brain with review of the literature. J Neurooncol. 25(2):155-9, 1995
                                                  47. 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

                                                                            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

                                                                                                • Image Interpretation Pearls

                                                                                                  Selected References

                                                                                                  1. Antonelli M et al: SMARCB1/INI1 involvement in pediatric chordoma: a mutational and immunohistochemical analysis. Am J Surg Pathol. 41(1):56-61, 2017
                                                                                                  2. Ramesh S et al: Multicentric chordoma in a child. J Pediatr Neurosci. 12(2):156-9, 2017
                                                                                                  3. Zhou J et al: Prognostic factors in patients with spinal chordoma: an integrative analysis of 682 patients. Neurosurgery. 81(5):812-23, 2017
                                                                                                  4. Sebro R et al: Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas. BMC Med Imaging. 16(1):53, 2016
                                                                                                  5. Tsitouras V et al: Management and outcome of chordomas in the pediatric population: the Hospital for Sick Children experience and review of the literature. J Clin Neurosci. 34:169-76, 2016
                                                                                                  6. Osaka S et al: Long-term outcome following surgical treatment of sacral chordoma. J Surg Oncol. 109(3):184-8, 2014
                                                                                                  7. 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
                                                                                                  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. Sciubba DM et al: Chordoma of the spinal column. Neurosurg Clin N Am. 19(1):5-15, 2008
                                                                                                  11. Takeyama J et al: Giant chordoma occupying the whole abdominal cavity. Pathology. 40(3):313-4, 2008
                                                                                                  12. Chugh R et al: Chordoma: the nonsarcoma primary bone tumor. Oncologist. 12(11):1344-50, 2007
                                                                                                  13. Boriani S et al: Chordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976). 31(4):493-503, 2006
                                                                                                  14. Takeyama J et al: Notochordal remnant-derived mass: ecchordosis physaliphora or chordoma? Pathology. 38(6):599-600, 2006
                                                                                                  15. Maclean FM et al: Chordoma: radiological-pathological correlation. Australas Radiol. 49(4):261-8, 2005
                                                                                                  16. Shinde SV et al: Craniospinal dissemination of clival chondroid chordoma. J Postgrad Med. 51(3):220-2, 2005
                                                                                                  17. 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
                                                                                                  18. 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
                                                                                                  19. Smolders D et al: Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol. 32(6):343-50, 2003
                                                                                                  20. Bayar MA et al: Spinal chordoma of the terminal filum. Case report. J Neurosurg. 96(2 Suppl):236-8, 2002
                                                                                                  21. 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
                                                                                                  22. Delank KS et al: Metastasizing chordoma of the lumbar spine. Eur Spine J. 11(2):167-71, 2002
                                                                                                  23. Leone A et al: Chordoma of the low cervical spine presenting with Horner's syndrome. Eur Radiol. 12 Suppl 3:S43-7, 2002
                                                                                                  24. Steenberghs J et al: Intradural chordoma without bone involvement. Case report and review of the literature. J Neurosurg. 97(1 Suppl):94-7, 2002
                                                                                                  25. Arnautovic KI et al: Surgical seeding of chordomas. J Neurosurg. 95(5):798-803, 2001
                                                                                                  26. Bosma JJ et al: En bloc removal of the lower lumbar vertebral body for chordoma. Report of two cases. J Neurosurg. 94(2 Suppl):284-91, 2001
                                                                                                  27. Crapanzano JP et al: Chordoma: a cytologic study with histologic and radiologic correlation. Cancer. 93(1):40-51, 2001
                                                                                                  28. McMaster ML et al: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 12(1):1-11, 2001
                                                                                                  29. 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
                                                                                                  30. Soo MY: Chordoma: review of clinicoradiological features and factors affecting survival. Australas Radiol. 45(4):427-34, 2001
                                                                                                  31. 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
                                                                                                  32. Tai PT et al: Management issues in chordoma: a case series. Clin Oncol (R Coll Radiol). 12(2):80-6, 2000
                                                                                                  33. Cheng EY et al: Lumbosacral chordoma. Prognostic factors and treatment. Spine. 24(16):1639-45, 1999
                                                                                                  34. Gelabert-Gonzalez M et al: Intradural cervical chordoma. Case report. J Neurosurg Sci. 43(2):159-62, 1999
                                                                                                  35. Wippold FJ 2nd et al: Clinical and imaging features of cervical chordoma. AJR Am J Roentgenol. 172(5):1423-6, 1999
                                                                                                  36. York JE et al: Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 44(1):74-9; discussion 79-80, 1999
                                                                                                  37. Logroscino CA et al: Chordoma: long-term evaluation of 15 cases treated surgically. Chir Organi Mov. 83(1-2):87-103, 1998
                                                                                                  38. Murphy JM et al: CT and MRI appearances of a thoracic chordoma. Eur Radiol. 8(9):1677-9, 1998
                                                                                                  39. Ng SH et al: Cervical ecchordosis physaliphora: CT and MR features. Br J Radiol. 71(843):329-31, 1998
                                                                                                  40. Boriani S et al: Primary bone tumors of the spine. Terminology and surgical staging. Spine. 22(9):1036-44, 1997
                                                                                                  41. Boriani S et al: Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine. 21(13):1569-77, 1996
                                                                                                  42. Catton C et al: Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol. 41(1):67-72, 1996
                                                                                                  43. Klekamp J et al: Spinal chordomas--results of treatment over a 17-year period. Acta Neurochir (Wien). 138(5):514-9, 1996
                                                                                                  44. Murphey MD et al: From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics. 16(5):1131-58, 1996
                                                                                                  45. 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
                                                                                                  46. Hall WA et al: Sacrococcygeal chordoma metastatic to the brain with review of the literature. J Neurooncol. 25(2):155-9, 1995
                                                                                                  47. 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