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Chordoma
Hilda E. Stambuk, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Clival chordoma (CCh)
          • Synonyms

            • Basicranial chordoma
          • Definitions

            • Rare, locally aggressive tumor of clivus arising from cranial end of primitive notochord remnant

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Angiographic 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

                                          • Reporting Tips

                                            Selected References

                                            1. Campbell RG et al: Contemporary management of clival chordomas. Curr Opin Otolaryngol Head Neck Surg. 23(2):153-61, 2015
                                            2. Choy W et al: Predictors of recurrence following resection of intracranial chordomas. J Clin Neurosci. 22(11):1792-6, 2015
                                            3. Kanamori H et al: Genetic characterization of skull base chondrosarcomas. J Neurosurg. 123(4):1036-41, 2015
                                            4. Müller U et al: Is there a role for conventional MRI and MR diffusion-weighted imaging for distinction of skull base chordoma and chondrosarcoma? Acta Radiol. ePub, 2015
                                            5. Policeni BA et al: Imaging of the skull base: anatomy and pathology. Radiol Clin North Am. 53(1):1-14, 2015
                                            6. Van Gompel JJ et al: Chordoma and chondrosarcoma. Otolaryngol Clin North Am. 48(3):501-14, 2015
                                            7. Amit M et al: Treatment and outcome of patients with skull base chordoma: a meta-analysis. J Neurol Surg B Skull Base. 75(6):383-90, 2014
                                            8. Bohman LE et al: Skull base chordoma and chondrosarcoma: influence of clinical and demographic factors on prognosis: a SEER analysis. World Neurosurg. 82(5):806-14, 2014
                                            9. Yeom KW et al: Diffusion-weighted MRI: distinction of skull base chordoma from chondrosarcoma. AJNR Am J Neuroradiol. 34(5):1056-61, S1, 2013
                                            10. Fraser JF et al: Endoscopic endonasal transclival resection of chordomas: operative technique, clinical outcome, and review of the literature. J Neurosurg. 112(5):1061-9, 2010
                                            11. Ares C et al: Effectiveness and safety of spot scanning proton radiation therapy for chordomas and chondrosarcomas of the skull base: first long-term report. Int J Radiat Oncol Biol Phys. 75(4):1111-8, 2009
                                            12. Stippler M et al: Endoscopic endonasal approach for clival chordomas. Neurosurgery. 64(2):268-77; discussion 277-8, 2009
                                            13. Takahashi S et al: Skull base chordomas: efficacy of surgery followed by carbon ion radiotherapy. Acta Neurochir (Wien). 151(7):759-69, 2009
                                            14. 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
                                            15. Yoneoka Y et al: Cranial base chordoma--long term outcome and review of the literature. Acta Neurochir (Wien). 150(8):773-8; discussion 778, 2008
                                            16. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                            17. Samii A et al: Chordomas of the skull base: surgical management and outcome. J Neurosurg. 107(2):319-24, 2007
                                            18. Pamir MN et al: Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas. Eur J Radiol. 58(3):461-70, 2006
                                            19. Géhanne C et al: Skull base chordoma: CT and MRI features. JBR-BTR. 88(6):325-7, 2005
                                            20. Noël G et al: Chordomas of the base of the skull and upper cervical spine. One hundred patients irradiated by a 3D conformal technique combining photon and proton beams. Acta Oncol. 44(7):700-8, 2005
                                            21. Asano S et al: Intradural spinal seeding of a clival chordoma. Acta Neurochir (Wien). 145(7):599-603; discussion 603, 2003
                                            22. Erdem E et al: Comprehensive review of intracranial chordoma. Radiographics. 23(4):995-1009, 2003
                                            23. Lanzino G et al: Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus. 10(3):E12, 2001
                                            24. Sen C et al: Cranial chordomas: results of radical excision. Neurosurg Focus. 10(3):E3, 2001
                                            25. Fischbein NJ et al: Recurrence of clival chordoma along the surgical pathway. AJNR Am J Neuroradiol. 21(3):578-83, 2000
                                            26. Hug EB et al: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. Neurosurg Clin N Am. 11(4):627-38, 2000
                                            27. Rosenberg AE et al: Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol. 23(11):1370-8, 1999
                                            28. Uggowitzer MM et al: Drop metastases in a patient with a chondroid chordoma of the clivus. Neuroradiology. 41(7):504-7, 1999
                                            29. Doucet V et al: MRI of intracranial chordomas. Extent of tumour and contrast enhancement: criteria for differential diagnosis. Neuroradiology. 39(8):571-6, 1997
                                            30. Harada T et al: Clival chordoma presenting as acute esotropia due to bilateral abducens palsy. Ophthalmologica. 211(2):109-11, 1997
                                            31. Ikushima I et al: Chordomas of the skull base: dynamic MRI. J Comput Assist Tomogr. 20(4):547-50, 1996
                                            32. Maira G et al: Surgical treatment of clival chordomas: the transsphenoidal approach revisited. J Neurosurg. 85(5):784-92, 1996
                                            33. Weber AL et al: Cartilaginous tumors and chordomas of the cranial base. Otolaryngol Clin North Am. 28(3):453-71, 1995
                                            34. Tashiro T et al: Intradural chordoma: case report and review of the literature. Neuroradiology. 36(4):313-5, 1994
                                            35. Weber AL et al: Chordomas of the skull base. Radiologic and clinical evaluation. Neuroimaging Clin N Am. 4(3):515-27, 1994
                                            36. Leproux F et al: MRI of cranial chordomas: the value of gadolinium. Neuroradiology. 35(7):543-5, 1993
                                            37. Probst EN et al: Congenital clivus chordoma. AJNR Am J Neuroradiol. 14(3):537-9, 1993
                                            38. Schamschula RG et al: Clival chordomas. Australas Radiol. 37(3):259-64, 1993
                                            39. Meyers SP et al: Chordomas of the skull base: MR features. AJNR Am J Neuroradiol. 13(6):1627-36, 1992
                                            40. Brown RV et al: CT and MR findings in patients with chordomas of the petrous apex. AJNR Am J Neuroradiol. 11(1):121-4, 1990
                                            41. Woźnica J et al: Value of CT and NMR imaging in diagnosing of chordomas. Ann Univ Mariae Curie Sklodowska [Med]. 45:181-6, 1990
                                            42. Oot RF et al: The role of MR and CT in evaluating clival chordomas and chondrosarcomas. AJR Am J Roentgenol. 151(3):567-75, 1988
                                            43. Sze G et al: Chordomas: MR imaging. Radiology. 166(1 Pt 1):187-91, 1988
                                            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

                                                    • Abbreviations

                                                      • Clival chordoma (CCh)
                                                    • Synonyms

                                                      • Basicranial chordoma
                                                    • Definitions

                                                      • Rare, locally aggressive tumor of clivus arising from cranial end of primitive notochord remnant

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Angiographic 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

                                                                                    • Reporting Tips

                                                                                      Selected References

                                                                                      1. Campbell RG et al: Contemporary management of clival chordomas. Curr Opin Otolaryngol Head Neck Surg. 23(2):153-61, 2015
                                                                                      2. Choy W et al: Predictors of recurrence following resection of intracranial chordomas. J Clin Neurosci. 22(11):1792-6, 2015
                                                                                      3. Kanamori H et al: Genetic characterization of skull base chondrosarcomas. J Neurosurg. 123(4):1036-41, 2015
                                                                                      4. Müller U et al: Is there a role for conventional MRI and MR diffusion-weighted imaging for distinction of skull base chordoma and chondrosarcoma? Acta Radiol. ePub, 2015
                                                                                      5. Policeni BA et al: Imaging of the skull base: anatomy and pathology. Radiol Clin North Am. 53(1):1-14, 2015
                                                                                      6. Van Gompel JJ et al: Chordoma and chondrosarcoma. Otolaryngol Clin North Am. 48(3):501-14, 2015
                                                                                      7. Amit M et al: Treatment and outcome of patients with skull base chordoma: a meta-analysis. J Neurol Surg B Skull Base. 75(6):383-90, 2014
                                                                                      8. Bohman LE et al: Skull base chordoma and chondrosarcoma: influence of clinical and demographic factors on prognosis: a SEER analysis. World Neurosurg. 82(5):806-14, 2014
                                                                                      9. Yeom KW et al: Diffusion-weighted MRI: distinction of skull base chordoma from chondrosarcoma. AJNR Am J Neuroradiol. 34(5):1056-61, S1, 2013
                                                                                      10. Fraser JF et al: Endoscopic endonasal transclival resection of chordomas: operative technique, clinical outcome, and review of the literature. J Neurosurg. 112(5):1061-9, 2010
                                                                                      11. Ares C et al: Effectiveness and safety of spot scanning proton radiation therapy for chordomas and chondrosarcomas of the skull base: first long-term report. Int J Radiat Oncol Biol Phys. 75(4):1111-8, 2009
                                                                                      12. Stippler M et al: Endoscopic endonasal approach for clival chordomas. Neurosurgery. 64(2):268-77; discussion 277-8, 2009
                                                                                      13. Takahashi S et al: Skull base chordomas: efficacy of surgery followed by carbon ion radiotherapy. Acta Neurochir (Wien). 151(7):759-69, 2009
                                                                                      14. 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
                                                                                      15. Yoneoka Y et al: Cranial base chordoma--long term outcome and review of the literature. Acta Neurochir (Wien). 150(8):773-8; discussion 778, 2008
                                                                                      16. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                                                                      17. Samii A et al: Chordomas of the skull base: surgical management and outcome. J Neurosurg. 107(2):319-24, 2007
                                                                                      18. Pamir MN et al: Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas. Eur J Radiol. 58(3):461-70, 2006
                                                                                      19. Géhanne C et al: Skull base chordoma: CT and MRI features. JBR-BTR. 88(6):325-7, 2005
                                                                                      20. Noël G et al: Chordomas of the base of the skull and upper cervical spine. One hundred patients irradiated by a 3D conformal technique combining photon and proton beams. Acta Oncol. 44(7):700-8, 2005
                                                                                      21. Asano S et al: Intradural spinal seeding of a clival chordoma. Acta Neurochir (Wien). 145(7):599-603; discussion 603, 2003
                                                                                      22. Erdem E et al: Comprehensive review of intracranial chordoma. Radiographics. 23(4):995-1009, 2003
                                                                                      23. Lanzino G et al: Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus. 10(3):E12, 2001
                                                                                      24. Sen C et al: Cranial chordomas: results of radical excision. Neurosurg Focus. 10(3):E3, 2001
                                                                                      25. Fischbein NJ et al: Recurrence of clival chordoma along the surgical pathway. AJNR Am J Neuroradiol. 21(3):578-83, 2000
                                                                                      26. Hug EB et al: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. Neurosurg Clin N Am. 11(4):627-38, 2000
                                                                                      27. Rosenberg AE et al: Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol. 23(11):1370-8, 1999
                                                                                      28. Uggowitzer MM et al: Drop metastases in a patient with a chondroid chordoma of the clivus. Neuroradiology. 41(7):504-7, 1999
                                                                                      29. Doucet V et al: MRI of intracranial chordomas. Extent of tumour and contrast enhancement: criteria for differential diagnosis. Neuroradiology. 39(8):571-6, 1997
                                                                                      30. Harada T et al: Clival chordoma presenting as acute esotropia due to bilateral abducens palsy. Ophthalmologica. 211(2):109-11, 1997
                                                                                      31. Ikushima I et al: Chordomas of the skull base: dynamic MRI. J Comput Assist Tomogr. 20(4):547-50, 1996
                                                                                      32. Maira G et al: Surgical treatment of clival chordomas: the transsphenoidal approach revisited. J Neurosurg. 85(5):784-92, 1996
                                                                                      33. Weber AL et al: Cartilaginous tumors and chordomas of the cranial base. Otolaryngol Clin North Am. 28(3):453-71, 1995
                                                                                      34. Tashiro T et al: Intradural chordoma: case report and review of the literature. Neuroradiology. 36(4):313-5, 1994
                                                                                      35. Weber AL et al: Chordomas of the skull base. Radiologic and clinical evaluation. Neuroimaging Clin N Am. 4(3):515-27, 1994
                                                                                      36. Leproux F et al: MRI of cranial chordomas: the value of gadolinium. Neuroradiology. 35(7):543-5, 1993
                                                                                      37. Probst EN et al: Congenital clivus chordoma. AJNR Am J Neuroradiol. 14(3):537-9, 1993
                                                                                      38. Schamschula RG et al: Clival chordomas. Australas Radiol. 37(3):259-64, 1993
                                                                                      39. Meyers SP et al: Chordomas of the skull base: MR features. AJNR Am J Neuroradiol. 13(6):1627-36, 1992
                                                                                      40. Brown RV et al: CT and MR findings in patients with chordomas of the petrous apex. AJNR Am J Neuroradiol. 11(1):121-4, 1990
                                                                                      41. Woźnica J et al: Value of CT and NMR imaging in diagnosing of chordomas. Ann Univ Mariae Curie Sklodowska [Med]. 45:181-6, 1990
                                                                                      42. Oot RF et al: The role of MR and CT in evaluating clival chordomas and chondrosarcomas. AJR Am J Roentgenol. 151(3):567-75, 1988
                                                                                      43. Sze G et al: Chordomas: MR imaging. Radiology. 166(1 Pt 1):187-91, 1988