Skip to main content
SpringerLink
Log in

Introduction

  • Chapter
Midline Skull Base Surgery

  • 1408 Accesses

Abstract

Chordomas are quite rare pathological entities occurring with an overall incidence rate of 0.08 per 100,000 [24, 26, 44, 47, 49]. They may occur at any age, although they are most commonly encountered in male patients during the third, fourth, and fifth decades of life and rarely appear in children (<5 %). Chordomas histologically derive from remnants of embryonic notochordal tissue a rodlike cord of cells from which the skull base and vertebral column develop [26, 32, 44, 46]; indeed, they can arise anywhere along the axial skeleton – nearly always within the bone – having a predilection for the sacrum, clivus, or cervical vertebrae [24, 26, 32, 44, 47, 57]. In rare cases (3–7 %), they arise from the cervical spine (cervical chordomas), presenting as a paravertebral or parapharyngeal mass [24, 26, 42, 44, 64].

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 149.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Al-Mefty O, Borba LA (1997) Skull base chordomas: a management challenge. J Neurosurg 86(2):182–189. doi:10.3171/jns.1997.86.2.0182

    Article  CAS  PubMed  Google Scholar 

  2. Al-Mefty O, Kadri PA, Hasan DM, Isolan GR, Pravdenkova S (2008) Anterior clivectomy: surgical technique and clinical applications. J Neurosurg 109(5):783–793. doi:10.3171/JNS/2008/109/11/0783

    Article  PubMed  Google Scholar 

  3. Anand VK, Harkey HL, Al-Mefty O (1991) Open-door maxillotomy approach for lesions of the clivus. Skull Base Surg 1(4):217–225

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  4. Ares C, Hug EB, Lomax AJ, Bolsi A, Timmermann B, Rutz HP, Schuller JC, Pedroni E, Goitein G (2009) 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):1111–1118. doi:10.1016/j.ijrobp.2008.12.055

    Article  PubMed  Google Scholar 

  5. Barnes L, Kapadia SB (1994) The biology and pathology of selected skull base tumors. J Neurooncol 20(3):213–240

    Article  CAS  PubMed  Google Scholar 

  6. Bertalanffy H, Seeger W (1991) The dorsolateral, suboccipital, transcondylar approach to the lower clivus and anterior portion of the craniocervical junction. Neurosurgery 29(6):815–821

    Article  CAS  PubMed  Google Scholar 

  7. Borba LA, Al-Mefty O, Mrak RE, Suen J (1996) Cranial chordomas in children and adolescents. J Neurosurg 84(4):584–591. doi:10.3171/jns.1996.84.4.0584

    Article  CAS  PubMed  Google Scholar 

  8. Cappabianca P, Cavallo LM, Esposito F, de Divitiis O, Messina A, de Divitiis E (2008) Extended endoscopic endonasal approach to the midline skull base: the evolving role of transsphenoidal surgery. In: Pickard JD, Akalan N, Di Rocco C et al (eds) Advances and technical standards in neurosurgery. Springer, Wien/New York, pp 152–199

    Google Scholar 

  9. Catton C, O’Sullivan B, Bell R, Laperriere N, Cummings B, Fornasier V, Wunder J (1996) Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol 41(1):67–72

    Article  CAS  PubMed  Google Scholar 

  10. Chibbaro S, Cornelius JF, Froelich S, Tigan L, Kehrli P, Debry C, Romano A, Herman P, George B, Bresson D (2014) Endoscopic endonasal approach in the management of skull base chordomas – clinical experience on a large series, technique, outcome, and pitfalls. Neurosurg Rev 37(2):217–224; discussion 224–215. doi:10.1007/s10143-013-0503-9

    Google Scholar 

  11. Choi D, Crockard HA (2013) Evolution of transoral surgery: three decades of change in patients, pathologies, and indications. Neurosurgery 73(2):296–303; discussion 303–294. doi:10.1227/01.neu.0000430324.24623.10

    Google Scholar 

  12. Ciporen JN, Moe KS, Ramanathan D, Lopez S, Ledesma E, Rostomily R, Sekhar LN (2010) Multiportal endoscopic approaches to the central skull base: a cadaveric study. World Neurosurg 73(6):705–712. doi:10.1016/j.wneu.2010.03.033

    Article  PubMed  Google Scholar 

  13. Cocke EW Jr, Robertson JH, Robertson JT, Crook JP Jr (1990) The extended maxillotomy and subtotal maxillectomy for excision of skull base tumors. Arch Otolaryngol Head Neck Surg 116(1):92–104

    Article  PubMed  Google Scholar 

  14. Colli BO, Al-Mefty O (2001) Chordomas of the skull base: follow-up review and prognostic factors. Neurosurg Focus 10(3), E1. doi:10.3171/foc.2001.10.3.2

    Article  CAS  PubMed  Google Scholar 

  15. Couldwell WT, Weiss MH, Rabb C, Liu JK, Apfelbaum RI, Fukushima T (2004) Variations on the standard transsphenoidal approach to the sellar region, with emphasis on the extended approaches and parasellar approaches: surgical experience in 105 cases. Neurosurgery 55(3):539–550

    Article  PubMed  Google Scholar 

  16. Crockard HA, Steel T, Plowman N, Singh A, Crossman J, Revesz T, Holton JL, Cheeseman A (2001) A multidisciplinary team approach to skull base chordomas. J Neurosurg 95(2):175–183. doi:10.3171/jns.2001.95.2.0175

    Article  CAS  PubMed  Google Scholar 

  17. Crumley RL, Gutin PH (1989) Surgical access for clivus chordoma. The University of California, San Francisco, experience. Arch Otolaryngol Head Neck Surg 115(3):295–300

    Article  CAS  PubMed  Google Scholar 

  18. de Divitiis E, Cappabianca P, Cavallo LM (2002) Endoscopic transsphenoidal approach: adaptability of the procedure to different sellar lesions. Neurosurgery 51(3):699–705; discussion 705–707

    PubMed  Google Scholar 

  19. Delgado TE, Garrido E, Harwick RD (1981) Labiomandibular, transoral approach to chordomas in the clivus and upper cervical spine. Neurosurgery 8(6):675–679

    Article  CAS  PubMed  Google Scholar 

  20. DeMonte F, Diaz E Jr, Callender D, Suk I (2001) Transmandibular, circumglossal, retropharyngeal approach for chordomas of the clivus and upper cervical spine. Technical note. Neurosurg Focus 10(3), E10

    Article  CAS  PubMed  Google Scholar 

  21. Diaz RJ, Cusimano MD (2011) The biological basis for modern treatment of chordoma. J Neurooncol 104(2):411–422. doi:10.1007/s11060-011-0559-8

    Article  PubMed  Google Scholar 

  22. Diaz RJ, Guduk M, Romagnuolo R, Smith CA, Northcott P, Shih D, Berisha F, Flanagan A, Munoz DG, Cusimano MD, Pamir MN, Rutka JT (2012) High-resolution whole-genome analysis of skull base chordomas implicates FHIT loss in chordoma pathogenesis. Neoplasia 14(9):788–798

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  23. Diaz RJ, Maggacis N, Zhang S, Cusimano MD (2014) Determinants of quality of life in patients with skull base chordoma. J Neurosurg 120(2):528–537. doi:10.3171/2013.9.JNS13671

    Article  PubMed  Google Scholar 

  24. Eriksson B, Gunterberg B, Kindblom LG (1981) Chordoma. A clinicopathologic and prognostic study of a Swedish national series. Acta Orthop Scand 52(1):49–58

    Article  CAS  PubMed  Google Scholar 

  25. Fatemi N, Dusick JR, Gorgulho AA, Mattozo CA, Moftakhar P, De Salles AA, Kelly DF (2008) Endonasal microscopic removal of clival chordomas. Surg Neurol 69(4):331–338. doi:10.1016/j.surneu.2007.08.035, S0090-3019(07)00985-8 [pii]

    Article  PubMed  Google Scholar 

  26. Fernandez-Miranda JC, Gardner PA, Snyderman CH, Devaney KO, Mendenhall WM, Suarez C, Rinaldo A, Ferlito A (2014) Clival chordomas: a pathological, surgical, and radiotherapeutic review. Head Neck 36(6):892–906. doi:10.1002/hed.23415

    Article  PubMed  Google Scholar 

  27. Feuvret L, Noel G, Weber DC, Pommier P, Ferrand R, De Marzi L, Dhermain F, Alapetite C, Mammar H, Boisserie G, Habrand JL, Mazeron JJ (2007) A treatment planning comparison of combined photon-proton beams versus proton beams-only for the treatment of skull base tumors. Int J Radiat Oncol Biol Phys 69(3):944–954. doi:10.1016/j.ijrobp.2007.07.2326

    Article  PubMed  Google Scholar 

  28. Frank G, Sciarretta V, Calbucci F, Farneti G, Mazzatenta D, Pasquini E (2006) The endoscopic transnasal transsphenoidal approach for the treatment of cranial base chordomas and chondrosarcomas. Neurosurgery 59(1 Suppl 1):ONS50–ONS57; discussion ONS50–57. doi:10.1227/01.NEU.0000219914.17221.55

    Google Scholar 

  29. Fraser JF, Nyquist GG, Moore N, Anand VK, Schwartz TH (2010) Endoscopic endonasal transclival resection of chordomas: operative technique, clinical outcome, and review of the literature. J Neurosurg 112(5):1061–1069. doi:10.3171/2009.7.JNS081504

    Article  PubMed  Google Scholar 

  30. Hardy J, Vezina JL (1976) Transsphenoidal neurosurgery of intracranial neoplasm. Adv Neurol 15:261–273

    CAS  PubMed  Google Scholar 

  31. Harsh GR, Sekhar LN (1992) The subtemporal, transcavernous, anterior transpetrosal approach to the upper brain stem and clivus. J Neurosurg 77(5):709–717. doi:10.3171/jns.1992.77.5.0709

    Article  PubMed  Google Scholar 

  32. Hoch BL, Nielsen GP, Liebsch NJ, Rosenberg AE (2006) Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 30(7):811–818. doi:10.1097/01.pas.0000209828.39477.ab

    Article  PubMed  Google Scholar 

  33. Igaki H, Tokuuye K, Okumura T, Sugahara S, Kagei K, Hata M, Ohara K, Hashimoto T, Tsuboi K, Takano S, Matsumura A, Akine Y (2004) Clinical results of proton beam therapy for skull base chordoma. Int J Radiat Oncol Biol Phys 60(4):1120–1126. doi:10.1016/j.ijrobp.2004.05.064

    Article  PubMed  Google Scholar 

  34. James D, Crockard HA (1991) Surgical access to the base of skull and upper cervical spine by extended maxillotomy. Neurosurgery 29(3):411–416

    Article  CAS  PubMed  Google Scholar 

  35. Janecka IP, Nuss DW, Sen CN (1991) Facial translocation approach to the cranial base. Acta Neurochir Suppl 53:193–198

    Article  CAS  PubMed  Google Scholar 

  36. Jeffrey PB, Biava CG, Davis RL (1995) Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation. Am J Clin Pathol 103(3):271–279

    CAS  PubMed  Google Scholar 

  37. Kassam A, Snyderman CH, Mintz A, Gardner P, Carrau RL (2005) Expanded endonasal approach: the rostrocaudal axis. Part I. Crista galli to the sella turcica. Neurosurg Focus 19(1):E3, 1–12

    PubMed  Google Scholar 

  38. Kassam A, Snyderman CH, Mintz A, Gardner P, Carrau RL (2005) Expanded endonasal approach: the rostrocaudal axis. Part II. Posterior clinoids to the foramen magnum. Neurosurg Focus 19(1), E4

    PubMed  Google Scholar 

  39. Kassam AB, Prevedello DM, Carrau RL, Snyderman CH, Thomas A, Gardner P, Zanation A, Duz B, Stefko ST, Byers K, Horowitz MB (2011) Endoscopic endonasal skull base surgery: analysis of complications in the authors’ initial 800 patients. J Neurosurg 114(6):1544–1568. doi:10.3171/2010.10.JNS09406

    Article  PubMed  Google Scholar 

  40. Komotar RJ, Starke RM, Raper DM, Anand VK, Schwartz TH (2011) The endoscope-assisted ventral approach compared with open microscope-assisted surgery for clival chordomas. World Neurosurg 76(3–4):318–327; discussion 259–362. doi:10.1016/j.wneu.2011.02.026. S1878-8750(11)00145-8 [pii]

    Google Scholar 

  41. Koutourousiou M, Gardner PA, Tormenti MJ, Henry SL, Stefko ST, Kassam AB, Fernandez-Miranda JC, Snyderman CH (2012) Endoscopic endonasal approach for resection of cranial base chordomas: outcomes and learning curve. Neurosurgery 71(3):614–624; discussion 624–615. doi:10.1227/NEU.0b013e31825ea3e0

    Google Scholar 

  42. Lanzino G, Dumont AS, Lopes MB, Laws ER Jr (2001) Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus 10(3), E12

    Article  CAS  PubMed  Google Scholar 

  43. Laufer I, Anand VK, Schwartz TH (2007) Endoscopic, endonasal extended transsphenoidal, transplanum transtuberculum approach for resection of suprasellar lesions. J Neurosurg 106(3):400–406

    Article  PubMed  Google Scholar 

  44. Laws E (1993) Clivus chordomas. In: Sekhar LN, Janecka IP (eds) Surgery of cranial base tumors. Raven Press, New York, pp 679–685

    Google Scholar 

  45. Liu JK, Decker D, Schaefer SD, Moscatello AL, Orlandi RR, Weiss MH, Couldwell WT (2003) Zones of approach for craniofacial resection: minimizing facial incisions for resection of anterior cranial base and paranasal sinus tumors. Neurosurgery 53(5):1126–1135; discussion 1135–1127

    Article  PubMed  Google Scholar 

  46. Maira G, Pallini R, Anile C, Fernandez E, Salvinelli F, La Rocca LM, Rossi GF (1996) Surgical treatment of clival chordomas: the transsphenoidal approach revisited. J Neurosurg 85(5):784–792

    Article  CAS  PubMed  Google Scholar 

  47. McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM (2001) Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 12(1):1–11

    Article  CAS  PubMed  Google Scholar 

  48. Mendenhall NP, Malyapa RS, Su Z, Yeung D, Mendenhall WM, Li Z (2011) Proton therapy for head and neck cancer: rationale, potential indications, practical considerations, and current clinical evidence. Acta Oncol 50(6):763–771. doi:10.3109/0284186X.2011.590147

    Article  PubMed  Google Scholar 

  49. Mendenhall WM, Mendenhall CM, Lewis SB, Villaret DB, Mendenhall NP (2005) Skull base chordoma. Head Neck 27(2):159–165. doi:10.1002/hed.20144

    Article  PubMed  Google Scholar 

  50. Mitchell A, Scheithauer BW, Unni KK, Forsyth PJ, Wold LE, McGivney DJ (1993) Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer 72(10):2943–2949

    Article  CAS  PubMed  Google Scholar 

  51. O’Connell JX, Renard LG, Liebsch NJ, Efird JT, Munzenrider JE, Rosenberg AE (1994) Base of skull chordoma. A correlative study of histologic and clinical features of 62 cases. Cancer 74(8):2261–2267

    Article  PubMed  Google Scholar 

  52. 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(4):812–822. doi:10.3171/jns.2003.98.4.0812

    Article  PubMed  Google Scholar 

  53. Prevedello DM, Ditzel Filho LF, Solari D, Carrau RL, Kassam AB (2010) Expanded endonasal approaches to middle cranial fossa and posterior fossa tumors. Neurosurg Clin N Am 21(4):621–635. doi:10.1016/j.nec.2010.07.003, vi. S1042-3680(10)00059-8 [pii]

    Article  PubMed  Google Scholar 

  54. Rosenberg AE, Brown GA, Bhan AK, Lee JM (1994) Chondroid chordoma--a variant of chordoma. A morphologic and immunohistochemical study. Am J Clin Pathol 101(1):36–41

    CAS  PubMed  Google Scholar 

  55. Samii A, Gerganov VM, Herold C, Hayashi N, Naka T, Mirzayan MJ, Ostertag H, Samii M (2007) Chordomas of the skull base: surgical management and outcome. J Neurosurg 107(2):319–324. doi:10.3171/JNS-07/08/0319

    Article  PubMed  Google Scholar 

  56. Sen C, Triana A (2001) Cranial chordomas: results of radical excision. Neurosurg Focus 10(3), E3

    Article  CAS  PubMed  Google Scholar 

  57. Sen C, Triana AI, Berglind N, Godbold J, Shrivastava RK (2010) Clival chordomas: clinical management, results, and complications in 71 patients. J Neurosurg 113(5):1059–1071. doi:10.3171/2009.9.JNS08596

    Article  PubMed  Google Scholar 

  58. Srivastava A, Vischioni B, Fiore MR, Vitolo V, Fossati P, Iannalfi A, Tuan JK, Orecchia R (2013) Quality of life in patients with chordomas/chondrosarcomas during treatment with proton beam therapy. J Radiat Res 54(Suppl 1):i43–i48. doi:10.1093/jrr/rrt057

    Article  PubMed Central  PubMed  Google Scholar 

  59. Stippler M, Gardner PA, Snyderman CH, Carrau RL, Prevedello DM, Kassam AB (2009) Endoscopic endonasal approach for clival chordomas. Neurosurgery 64(2):268–277; discussion 277–268. doi:10.1227/01.NEU.0000338071.01241.E2. 00006123-200902000-00008 [pii]

    Google Scholar 

  60. Swearingen B, Joseph M, Cheney M, Ojemann RG (1995) A modified transfacial approach to the clivus. Neurosurgery 36(1):101–104; discussion 104–105

    Article  CAS  PubMed  Google Scholar 

  61. Tamaki N, Nagashima T, Ehara K, Motooka Y, Barua KK (2001) Surgical approaches and strategies for skull base chordomas. Neurosurg Focus 10(3), E9

    Article  CAS  PubMed  Google Scholar 

  62. 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(2):230–237; discussion 230–237. doi:10.1227/01.NEU.0000223441.51012.9D

    Google Scholar 

  63. Yasuda M, Bresson D, Chibbaro S, Cornelius JF, Polivka M, Feuvret L, Takayasu M, George B (2012) Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients. Neurosurg Rev 35(2):171–182; discussion 182–173. doi:10.1007/s10143-011-0334-5

    Google Scholar 

  64. Yoneoka Y, Tsumanuma I, Fukuda M, Tamura T, Morii K, Tanaka R, Fujii Y (2008) Cranial base chordoma – long term outcome and review of the literature. Acta Neurochir (Wien) 150(8):773–778; discussion 778. doi:10.1007/s00701-008-1600-3

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Neurosurgery, Department of Neurosciences, Reproductive and Odontostomatological Sciences, Università degli Studi di Napoli “Federico II”, Naples, Italy

    Paolo Cappabianca MD

  2. Anatomopathology Unit, Department of Advanced Biomedical Sciences, University of Naples “Federico II”, Naples, Italy

    Marialaura Del Basso De Caro BS

  3. Neuroradiology Unit, Department of Advanced Biomedical Sciences, Università degli Studi di Napoli “Federico II”, Naples, Italy

    Alessandra D’Amico MD

Authors
  1. Paolo Cappabianca MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Marialaura Del Basso De Caro BS
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Alessandra D’Amico MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Paolo Cappabianca MD .

Editor information

Editors and Affiliations

  1. Div. of Neurosurgery Dept. of Neuroscien, University of Naples “Federico II” , Naples, Italy

    Paolo Cappabianca

  2. Div. of Neurosurgery Dept. of Neuroscien, University of Naples “Federico II” , Naples, Italy

    Luigi Maria Cavallo

  3. Div. of Neurosurgery Dept. of Neuroscien, University of Naples “Federico II” , Naples, Italy

    Oreste de Divitiis

  4. Div. of Neurosurgery Dept. of Neuroscien, University of Messina , Messina, Messina, Italy

    Felice Esposito

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Cappabianca, P., De Caro, M.D., D’Amico, A. (2016). Introduction. In: Cappabianca, P., Cavallo, L., de Divitiis, O., Esposito, F. (eds) Midline Skull Base Surgery. Springer, Cham. https://doi.org/10.1007/978-3-319-21533-4_24

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-21533-4_24

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-21532-7

  • Online ISBN: 978-3-319-21533-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation