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Skull Base Chondrosarcoma
Philip R. Chapman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Chondrosarcoma-skull base (CSa-SB)
            • Definitions

              • CSa-SB: Chondroid malignancy of skull base

            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

                                            Selected References

                                            1. Awad M et al: Skull base chondrosarcoma. J Clin Neurosci. 24:1-5, 2016
                                            2. Bag AK et al: Neuroimaging: intrinsic lesions of the central skull base region. Semin Ultrasound CT MR. 34(5):412-35, 2013
                                            3. Bloch O et al: Skull base chondrosarcoma: evidence-based treatment paradigms. Neurosurg Clin N Am. 24(1):89-96, 2013
                                            4. Gardner PA et al: Carotid artery injury during endoscopic endonasal skull base surgery: incidence and outcomes. Neurosurgery. 73(2 Suppl Operative):ons261-9; discussion ons269-70, 2013
                                            5. Sbaihat A et al: Skull base chondrosarcomas: surgical treatment and results. Ann Otol Rhinol Laryngol. 122(12):763-70, 2013
                                            6. Amichetti M et al: A systematic review of proton therapy in the treatment of chondrosarcoma of the skull base. Neurosurg Rev. 33(2):155-65, 2010
                                            7. Bloch OG et al: Cranial chondrosarcoma and recurrence. Skull Base. 20(3):149-56, 2010
                                            8. 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
                                            9. Borges A: Imaging of the central skull base. Neuroimaging Clin N Am. 19(4):669-96, 2009
                                            10. Gallia GL et al: Skull base chondrosarcoma presenting with hemorrhage. Can J Neurol Sci. 36(6):774-5, 2009
                                            11. Güneş M et al: Intracranial chondrosarcoma: a case report and review of the literature. Minim Invasive Neurosurg. 52(5-6):238-41, 2009
                                            12. Hong P et al: Chondrosarcoma of the head and neck: report of 11 cases and literature review. J Otolaryngol Head Neck Surg. 38(2):279-85, 2009
                                            13. Samii A et al: Surgical treatment of skull base chondrosarcomas. Neurosurg Rev. 32(1):67-75; discussion 75, 2009
                                            14. Abdelmalek M et al: Recurrent chondrosarcoma of the right skull base in a patient with Maffucci syndrome. Am J Clin Dermatol. 9(1):61-5, 2008
                                            15. Cho YH et al: Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients. Neurosurg Rev. 31(1):35-43; discussion 43, 2008
                                            16. Ferrer Pérez AI et al: Skull base chondrosarcoma: a case treated by the CyberKnife system. Clin Transl Oncol. 10(10):670-2, 2008
                                            17. Gelderblom H et al: The clinical approach towards chondrosarcoma. Oncologist. 2008 Mar;13(3):320-9. Review. Erratum in: Oncologist. 13(5):618, 2008
                                            18. 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
                                            19. Sanna M et al: Chondrosarcomas of the jugular foramen. Laryngoscope. 118(10):1719-28, 2008
                                            20. Seizeur R et al: Chondrosarcoma of the skull base in Maffucci's syndrome. Br J Neurosurg. 22(6):778-80, 2008
                                            21. Zhang Q et al: Endoscopic endonasal surgery for clival chordoma and chondrosarcoma. ORL J Otorhinolaryngol Relat Spec. 70(2):124-9, 2008
                                            22. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                            23. Dini LI et al: Maffucci's syndrome complicated by intracranial chondrosarcoma: two new illustrative cases. Arq Neuropsiquiatr. 65(3B):816-21, 2007
                                            24. Lustig LR et al: Chondrosarcomas of the skull base and temporal bone. J Laryngol Otol. 121(8):725-35, 2007
                                            25. Schulz-Ertner D et al: Carbon ion radiotherapy of skull base chondrosarcomas. Int J Radiat Oncol Biol Phys. 67(1):171-7, 2007
                                            26. Baehring JM et al: Chondrosarcoma of the skull base. J Neurooncol. 76(1):49, 2006
                                            27. Brackmann DE et al: Chondrosarcoma of the skull base: long-term follow-up. Otol Neurotol. 27(7):981-91, 2006
                                            28. Frank G et al: The endoscopic transnasal transsphenoidal approach for the treatment of cranial base chordomas and chondrosarcomas. Neurosurgery. 59(1 Suppl 1):ONS50-7; discussion ONS50-7, 2006
                                            29. Harada K et al: Chondrosarcoma of the clivus presenting with intratumoral hemorrhage: case report. J Neurooncol. 77(2):221-3, 2006
                                            30. Tzortzidis F et al: Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chondrosarcomas. Neurosurgery. 58(6):1090-8; discussion 1090-8, 2006
                                            31. Krishnan S et al: Radiosurgery for cranial base chordomas and chondrosarcomas. Neurosurgery. 56(4):777-84; discussion 777-84, 2005
                                            32. Oghalai JS et al: Skull base chondrosarcoma originating from the petroclival junction. Otol Neurotol. 26(5):1052-60, 2005
                                            33. Noël G et al: Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome--three case reports and review of the literature. Acta Oncol. 43(8):705-10, 2004
                                            34. Raghu M et al: Chondrosarcomas of the temporal bone: presentation and management. J Laryngol Otol. 118(7):551-5, 2004
                                            35. Neff B et al: Chondrosarcoma of the skull base. Laryngoscope. 112(1):134-9, 2002
                                            36. Schmidinger A et al: Natural history of chondroid skull base lesions--case report and review. Neuroradiology. 44(3):268-71, 2002
                                            37. Crockard HA et al: A multidisciplinary team approach to skull base chondrosarcomas. J Neurosurg. 95(2):184-9, 2001
                                            38. Richardson MS: Pathology of skull base tumors. Otolaryngol Clin North Am. 34(6):1025-42, vii, 2001
                                            39. Hug EB et al: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg. 91(3):432-9, 1999
                                            40. 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
                                            41. Korten AG et al: Intracranial chondrosarcoma: review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry. 65(1):88-92, 1998
                                            42. Keel SB et al: Chondromyxoid fibroma of the skull base: a tumor which may be confused with chordoma and chondrosarcoma. A report of three cases and review of the literature. Am J Surg Pathol. 21(5):577-82, 1997
                                            43. Ramina R et al: Maffucci's syndrome associated with a cranial base chondrosarcoma: case report and literature review. Neurosurgery. 41(1):269-72, 1997
                                            44. Rapidis AD et al: Chondrosarcomas of the skull base: review of the literature and report of two cases. J Craniomaxillofac Surg. 25(6):322-7, 1997
                                            45. Geirnaerdt MJ et al: Calcified meningioma of the skull base simulating chondrosarcoma. Eur J Radiol. 21(2):148-51, 1995
                                            46. Weber AL et al: Cartilaginous tumors and chordomas of the cranial base. Otolaryngol Clin North Am. 28(3):453-71, 1995
                                            47. Brown E et al: Chondrosarcoma of the skull base. Neuroimaging Clin N Am. 4(3):529-41, 1994
                                            48. Stapleton SR et al: Chondrosarcoma of the skull base: a series of eight cases. Neurosurgery. 32(3):348-55; discussion 355-6, 1993
                                            49. Meyers SP et al: Chondrosarcomas of the skull base: MR imaging features. Radiology. 184(1):103-8, 1992
                                            50. Kveton JF et al: Chondrosarcoma of the skull base. Otolaryngol Head Neck Surg. 94(1):23-32, 1986
                                            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

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Chondrosarcoma-skull base (CSa-SB)
                                                      • Definitions

                                                        • CSa-SB: Chondroid malignancy of skull base

                                                      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

                                                                                      Selected References

                                                                                      1. Awad M et al: Skull base chondrosarcoma. J Clin Neurosci. 24:1-5, 2016
                                                                                      2. Bag AK et al: Neuroimaging: intrinsic lesions of the central skull base region. Semin Ultrasound CT MR. 34(5):412-35, 2013
                                                                                      3. Bloch O et al: Skull base chondrosarcoma: evidence-based treatment paradigms. Neurosurg Clin N Am. 24(1):89-96, 2013
                                                                                      4. Gardner PA et al: Carotid artery injury during endoscopic endonasal skull base surgery: incidence and outcomes. Neurosurgery. 73(2 Suppl Operative):ons261-9; discussion ons269-70, 2013
                                                                                      5. Sbaihat A et al: Skull base chondrosarcomas: surgical treatment and results. Ann Otol Rhinol Laryngol. 122(12):763-70, 2013
                                                                                      6. Amichetti M et al: A systematic review of proton therapy in the treatment of chondrosarcoma of the skull base. Neurosurg Rev. 33(2):155-65, 2010
                                                                                      7. Bloch OG et al: Cranial chondrosarcoma and recurrence. Skull Base. 20(3):149-56, 2010
                                                                                      8. 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
                                                                                      9. Borges A: Imaging of the central skull base. Neuroimaging Clin N Am. 19(4):669-96, 2009
                                                                                      10. Gallia GL et al: Skull base chondrosarcoma presenting with hemorrhage. Can J Neurol Sci. 36(6):774-5, 2009
                                                                                      11. Güneş M et al: Intracranial chondrosarcoma: a case report and review of the literature. Minim Invasive Neurosurg. 52(5-6):238-41, 2009
                                                                                      12. Hong P et al: Chondrosarcoma of the head and neck: report of 11 cases and literature review. J Otolaryngol Head Neck Surg. 38(2):279-85, 2009
                                                                                      13. Samii A et al: Surgical treatment of skull base chondrosarcomas. Neurosurg Rev. 32(1):67-75; discussion 75, 2009
                                                                                      14. Abdelmalek M et al: Recurrent chondrosarcoma of the right skull base in a patient with Maffucci syndrome. Am J Clin Dermatol. 9(1):61-5, 2008
                                                                                      15. Cho YH et al: Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients. Neurosurg Rev. 31(1):35-43; discussion 43, 2008
                                                                                      16. Ferrer Pérez AI et al: Skull base chondrosarcoma: a case treated by the CyberKnife system. Clin Transl Oncol. 10(10):670-2, 2008
                                                                                      17. Gelderblom H et al: The clinical approach towards chondrosarcoma. Oncologist. 2008 Mar;13(3):320-9. Review. Erratum in: Oncologist. 13(5):618, 2008
                                                                                      18. 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
                                                                                      19. Sanna M et al: Chondrosarcomas of the jugular foramen. Laryngoscope. 118(10):1719-28, 2008
                                                                                      20. Seizeur R et al: Chondrosarcoma of the skull base in Maffucci's syndrome. Br J Neurosurg. 22(6):778-80, 2008
                                                                                      21. Zhang Q et al: Endoscopic endonasal surgery for clival chordoma and chondrosarcoma. ORL J Otorhinolaryngol Relat Spec. 70(2):124-9, 2008
                                                                                      22. Almefty K et al: Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 110(11):2457-67, 2007
                                                                                      23. Dini LI et al: Maffucci's syndrome complicated by intracranial chondrosarcoma: two new illustrative cases. Arq Neuropsiquiatr. 65(3B):816-21, 2007
                                                                                      24. Lustig LR et al: Chondrosarcomas of the skull base and temporal bone. J Laryngol Otol. 121(8):725-35, 2007
                                                                                      25. Schulz-Ertner D et al: Carbon ion radiotherapy of skull base chondrosarcomas. Int J Radiat Oncol Biol Phys. 67(1):171-7, 2007
                                                                                      26. Baehring JM et al: Chondrosarcoma of the skull base. J Neurooncol. 76(1):49, 2006
                                                                                      27. Brackmann DE et al: Chondrosarcoma of the skull base: long-term follow-up. Otol Neurotol. 27(7):981-91, 2006
                                                                                      28. Frank G et al: The endoscopic transnasal transsphenoidal approach for the treatment of cranial base chordomas and chondrosarcomas. Neurosurgery. 59(1 Suppl 1):ONS50-7; discussion ONS50-7, 2006
                                                                                      29. Harada K et al: Chondrosarcoma of the clivus presenting with intratumoral hemorrhage: case report. J Neurooncol. 77(2):221-3, 2006
                                                                                      30. Tzortzidis F et al: Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chondrosarcomas. Neurosurgery. 58(6):1090-8; discussion 1090-8, 2006
                                                                                      31. Krishnan S et al: Radiosurgery for cranial base chordomas and chondrosarcomas. Neurosurgery. 56(4):777-84; discussion 777-84, 2005
                                                                                      32. Oghalai JS et al: Skull base chondrosarcoma originating from the petroclival junction. Otol Neurotol. 26(5):1052-60, 2005
                                                                                      33. Noël G et al: Chondrosarcomas of the base of the skull in Ollier's disease or Maffucci's syndrome--three case reports and review of the literature. Acta Oncol. 43(8):705-10, 2004
                                                                                      34. Raghu M et al: Chondrosarcomas of the temporal bone: presentation and management. J Laryngol Otol. 118(7):551-5, 2004
                                                                                      35. Neff B et al: Chondrosarcoma of the skull base. Laryngoscope. 112(1):134-9, 2002
                                                                                      36. Schmidinger A et al: Natural history of chondroid skull base lesions--case report and review. Neuroradiology. 44(3):268-71, 2002
                                                                                      37. Crockard HA et al: A multidisciplinary team approach to skull base chondrosarcomas. J Neurosurg. 95(2):184-9, 2001
                                                                                      38. Richardson MS: Pathology of skull base tumors. Otolaryngol Clin North Am. 34(6):1025-42, vii, 2001
                                                                                      39. Hug EB et al: Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg. 91(3):432-9, 1999
                                                                                      40. 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
                                                                                      41. Korten AG et al: Intracranial chondrosarcoma: review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry. 65(1):88-92, 1998
                                                                                      42. Keel SB et al: Chondromyxoid fibroma of the skull base: a tumor which may be confused with chordoma and chondrosarcoma. A report of three cases and review of the literature. Am J Surg Pathol. 21(5):577-82, 1997
                                                                                      43. Ramina R et al: Maffucci's syndrome associated with a cranial base chondrosarcoma: case report and literature review. Neurosurgery. 41(1):269-72, 1997
                                                                                      44. Rapidis AD et al: Chondrosarcomas of the skull base: review of the literature and report of two cases. J Craniomaxillofac Surg. 25(6):322-7, 1997
                                                                                      45. Geirnaerdt MJ et al: Calcified meningioma of the skull base simulating chondrosarcoma. Eur J Radiol. 21(2):148-51, 1995
                                                                                      46. Weber AL et al: Cartilaginous tumors and chordomas of the cranial base. Otolaryngol Clin North Am. 28(3):453-71, 1995
                                                                                      47. Brown E et al: Chondrosarcoma of the skull base. Neuroimaging Clin N Am. 4(3):529-41, 1994
                                                                                      48. Stapleton SR et al: Chondrosarcoma of the skull base: a series of eight cases. Neurosurgery. 32(3):348-55; discussion 355-6, 1993
                                                                                      49. Meyers SP et al: Chondrosarcomas of the skull base: MR imaging features. Radiology. 184(1):103-8, 1992
                                                                                      50. Kveton JF et al: Chondrosarcoma of the skull base. Otolaryngol Head Neck Surg. 94(1):23-32, 1986