Abstract
Chordomas are challenging tumours which usually occur in the axial skeleton, and are particularly difficult to manage when they are situated in the clivus and upper cervical spine due to the proximity of eloquent structures. They also have a tendency for local recurrence since enbloc resection is rarely possible in these locations. They have characteristic radiological appearances on MRI, and pathologically are characterised by the presence of Physallipherous cells. The mainstay of treatment is maximal tumour excision at the time of first presentation, followed by high dose radiation treatment such as proton beam irradiation.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Carpentier A, Polivka M, Blanquet A, Lot G, George B (2002) Suboccipital and cervical chordomas: the value of aggressive treatment at first presentation of the disease. J Neurosurg 97:1070–1077
Choi D, Melcher R, Harms J, Crockard A (2010) Outcome of 132 operations in 97 patients with chordomas of the craniocervical junction and upper cervical spine. Neurosurgery 61(1):59–65
Colli BO, Al-Mefty O (2001) Chordomas of the skull base: follow-up review and prognostic factors. Neurosurgery 95:933–943
Crockard HA (1985) The transoral approach to the base of the brain and upper cervical cord. Ann R Coll Surg Engl 67:321–325
Crockard HA, Johnston F (1993) Development of transoral approaches to lesions of the skull base and craniocervical junction. Neurosurg Q 3:61–82
Eriksson B, Gunterberg B, Kindblom LG (1981) Chordoma: a clinicopathological and prognostic study of a Swedish National series. Acta Orthop Scand 52:49–58
Forsyth PA, Cascino TL, Shaw EG, Scheithauer BW, O’Fallon JR, Dozier JC, Piepgras DG (1993) Intracranial chordomas: a clinicopathological and prognostic study of 51 cases. J Neurosurg 78:741–747
Foweraker KL, Burton KE, Maynard SE, Jena R, Jefferies SJ, Laing RJC, Burnet NG (2007) High-dose radiotherapy in the management of chordoma and chondrosarcoma of the skull base and cervical spine: part 1- clinical outcomes. Clin Oncol 19:509–516
Gay E, Sekhar LN, Rubinstein E, Wright DC, Sen C, Janecka IP, Snyderman CH (1995) Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:887–897
Greenberg AD, Scoville WB, Davey LM (1968) Transoral decompression of atlanto-axial dislocation due to odontoid hypoplasia. Report of two cases. J Neurosurg 28:266–269
Grison C (1967) Direct surgical approach by oral route to the first 2 cervical vertebrae. J Fr Otorhinolaryngol Audiophonol Chir Maxillofac 16:271–273
Harbour JW, Lawton MT, Criscuolo GR, Holliday MJ, Mattox DE, Long DM (1991) Clivus chordoma: a report of 12 recent cases and review of the literature. Skull Base Surg 1:200–206
Holton JL, Steel T, Luxsuwong M, Crockard HA, Revesz T (2000) Skull base chordomas: correlation of tumour doubling time with age, mitosis and Ki67 proliferation index. Neuropathol Appl Neurobiol 26:497–503
Hug EB, Loredo LN, Slater JD, DeVries A, Grove RI, Schaefer RA, Rosenberg AE, Slater JM (2000) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91:432–439
James D, Crockard HA (1991) Surgical access to the base of the skull and upper cervical spine by extended maxillotomy. Neurosurgery 29:411–416
Menezes AH, Van Gilder JC, Graf CJ, McDonnell DE (1980) Craniocervical abnormalities. A comprehensive surgical approach. J Neurosurg 53:444–455
Pallini R, Maira G, Pierconti F, Falchetti ML, Alvino E, Cimino-Reale G, Fernandez E, D’Ambrosio E, Larocca LM (2003) Chordoma of the skull base: predictors of tumor recurrence. J Neurosurg 98:1256–1262
Rock PJ, Tomecek FJ, Ross L (1993) Transoral surgery: an anatomic study. Skull Base Surg 3(3):109–116
Tzortzidis F, Elahi F, Wright D, Natarajan SK, Sekhar LN (2006) Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 59:230–237
Watkins L, Khudados ES, Kaleoglu M, Revesz T, Sacares P, Crockard HA (1993) Skull base chordomas: a review of 38 patients, 1958-88. Br J Neurosurg 7:241–248
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2012 Springer Science+Business Media B.V.
About this chapter
Cite this chapter
Choi, D. (2012). Chordomas of the Clivus and Upper Cervical Spine. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 6. Tumors of the Central Nervous System, vol 6. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-2866-0_11
Download citation
DOI: https://doi.org/10.1007/978-94-007-2866-0_11
Published:
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-007-2865-3
Online ISBN: 978-94-007-2866-0
eBook Packages: MedicineMedicine (R0)