Skip to main content

Chordomas and Chondrosarcomas

  • Chapter
  • First Online:
Samii's Essentials in Neurosurgery

Abstract

Chordomas and chondrosarcomas are rare primary tumors of the skull base, which are aggressive, are locally invasive, and have a high tendency to recur. Chordomas are believed to arise from remnants of the notochord that remain entrapped by bone. Chondrosarcomas are, on the other hand, thought to originate from fetal cartilage rests. Both tumors are generally discussed together due to overlapping clinical and imaging features. Histological differentiation is nowadays mandatory because current data have demonstrated a better survival for chondrosarcomas. The standard of care is cytoreductive surgery and postoperative radiotherapy. The rationale of management at our department is the utmost surgical resection, notwithstanding with minimal surgical morbidity preserving patients’ clinical status followed by adjuvant carbon ion radiotherapy in cases of residual tumors not scheduled for surgery or recurrent tumors. Palliative resections are planned in patients with recurrent tumors, and such patients may be considered for reirradiation.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
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 86:182–189

    Article  CAS  PubMed  Google Scholar 

  2. Al-Rahawan MM, Siebert JD, Mitchell CS, Smith SD (2012) Durable complete response to chemotherapy in an infant with a clival chordoma. Pediatr Blood Cancer 59:323–325

    Article  PubMed  Google Scholar 

  3. Arnautovic KI, Al-Mefty O (2001) Surgical seeding of chordomas. Neurosurg Focus 10:E7

    CAS  PubMed  Google Scholar 

  4. Austin JP, Urie MM, Cardenosa G, Munzenrider JE (1993) Probable causes of recurrence in patients with chordoma and chondrosarcoma of the base of skull and cervical spine. Int J Radiat Oncol Biol Phys 25:439–444

    Article  CAS  PubMed  Google Scholar 

  5. Bahr AL, Gayler BW (1977) Cranial chondrosarcomas. Report of four cases and review of the literature. Radiology 124:151–156

    CAS  PubMed  Google Scholar 

  6. Berghaus A, Jovanovic S (1991) Technique and indications of extended sublabial rhinotomy (“midfacial degloving”). Rhinology 29:105–110

    CAS  PubMed  Google Scholar 

  7. Berghaus A (1990) [Midfacial degloving]. HNO 38:7–11 (German)

    CAS  PubMed  Google Scholar 

  8. Boemke F, Joest W (1936) Chordome im Bereich des Schädels. Virchows Arch 297:351–367

    Article  Google Scholar 

  9. Chang SD, Martin DP, Lee E, Adler JR Jr (2001) Stereotactic radiosurgery and hypofractionated stereotactic radiotherapy for residual or recurrent cranial base and cervical chordomas. Neurosurg Focus 10:E5

    Article  CAS  PubMed  Google Scholar 

  10. Colli BO, Al-Mefty O (2001) Chordomas of the skull base: follow-up review and prognostic factors. Neurosurg Focus 10:E1

    Article  CAS  PubMed  Google Scholar 

  11. Combs SE, Kalbe A, Nikoghosyan A et al (2011) Carbon ion radiotherapy performed as re-irradiation using active beam delivery in patients with tumors of the brain, skull base and sacral region. Radiother Oncol 98:63–67

    Article  PubMed  Google Scholar 

  12. Crockard HA, Cheeseman A, Steel T, Revesz T, Holton JL, Plowman N et al (2001) A multidisciplinary team approach to skull base chondrosarcomas. J Neurosurg 95:184–189

    Article  CAS  PubMed  Google Scholar 

  13. Crockard HA, Steel T, Plowman N, Singh A, Crossman J, Revesz T et al (2001) A multidisciplinary team approach to skull base chordomas. J Neurosurg 95:175–183

    Article  CAS  PubMed  Google Scholar 

  14. Debus J, Schulz-Ertner D, Schad L, Essig M, Rhein B, Thillmann CO et al (2000) Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47:591–596

    Article  CAS  PubMed  Google Scholar 

  15. Dhall G, Traverso M, Finlay JL, Shane L, Gonzalez-Gomez I, Jubran R (2011) The role of chemotherapy in pediatric clival chordomas. J Neurooncol 103:657–662

    Article  CAS  PubMed  Google Scholar 

  16. Erdem E, Angtuaco EC, Van Hemert R, Park JS, Al-Mefty O (2003) Comprehensive review of intracranial chordoma. Radiographics 23:995–1009

    Article  PubMed  Google Scholar 

  17. Evans HL, Ayala AG, Romsdahl MM (1977) Prognostic factors in chondrosarcoma of bone: a clinicopathologic analysis with emphasis on histologic grading. Cancer 40:818–831

    Article  CAS  PubMed  Google Scholar 

  18. Feigl GC, Bundschuh O, Gharabaghi A, Safavi-Abassi S, El Shawarby A, Samii M et al (2005) Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas. J Neurosurg 102(Suppl):165–170

    Article  PubMed  Google Scholar 

  19. 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

    PubMed  Google Scholar 

  20. 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 112:1061–1069

    Article  PubMed  Google Scholar 

  21. Gay E, Sekhar LN, Rubinstein E, Wright DC, Sen C, Janecka IP et al (1995) Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:887–897

    Article  CAS  PubMed  Google Scholar 

  22. Gerszten PC, Pollack IF, Hamilton RL (1998) Primary parafalcine chondrosarcoma in a child. Acta Neuropathol (Berl) 95:111–114

    Article  CAS  Google Scholar 

  23. Griffith HB, Veerapen R (1987) A direct transnasal approach to the sphenoid sinus. Technical note. J Neurosurg 66:140–142

    Article  CAS  PubMed  Google Scholar 

  24. Haeckel C, Krueger S, Kuester D, Ostertag H, Samii M, Buehling F et al (2000) Expression of cathepsin K in chordoma. Hum Pathol 31:834–840

    Article  CAS  PubMed  Google Scholar 

  25. Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW (1973) Chordomas and cartilaginous tumors at the skull base. Cancer 32:410–420

    Article  CAS  PubMed  Google Scholar 

  26. 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 30:811–818

    Article  PubMed  Google Scholar 

  27. Horbinski C, Oakley GJ, Cieply K et al (2010) The prognostic value of Ki-67, p53, epidermal growth factor receptor, 1p36, 9p21, 10q23, and 17p13 in skull base chordomas. Arch Pathol Lab Med 134:1170–1176

    PubMed  Google Scholar 

  28. Horten BC, Montague SR (1976) Human ecchordosis physaliphora and chick embryonic notochord. A comparative electron microscopic study. Virchows Arch A Pathol Anat Histol 371:295–303

    Article  CAS  PubMed  Google Scholar 

  29. Hug EB, Loredo LN, Slater JD, DeVries A, Grove RI, Schaefer RA et al (1999) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91:432–439

    Article  CAS  PubMed  Google Scholar 

  30. Iyer A, Kano H, Kondziolka D et al (2012) Stereotactic radiosurgery for intracranial chondrosarcoma. J Neurooncol 108:535–542

    Article  PubMed  Google Scholar 

  31. Jho HD, Carrau RL, McLaughlin MR, Somaza SC (1997) Endoscopic transsphenoidal resection of a large chordoma in the posterior fossa. Acta Neurochir (Wien) 139:343–348

    Article  CAS  Google Scholar 

  32. 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 76:318–327

    Article  PubMed  Google Scholar 

  33. Kondziolka D, Lunsford LD, Flickinger JC (1991) The role of radiosurgery in the management of chordoma and chondrosarcoma of the cranial base. Neurosurgery 29:38–46

    Article  CAS  PubMed  Google Scholar 

  34. Korten AG, ter Berg HJ, Spincemaille GH, van der Laan RT, Van de Wel AM (1998) Intracranial chondrosarcoma: review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry 65:88–92

    Article  CAS  PubMed Central  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Google Scholar 

  37. Lichtenstein L, Bernstein D (1959) Unusual benign and malignant chondroid tumors of bone. A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumors, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer 12:1142–1157

    Article  Google Scholar 

  38. Lüdemann W, Samii M (2006) Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 59:237

    Google Scholar 

  39. Maier W, Lohnstein PU, Rosahl SK, Tatagiba M, Schipper J (2006) [Multidisciplinary management of skull base and craniocervical chordoma]. Laryngorhinootologie 85:272–278 (German)

    Article  CAS  PubMed  Google Scholar 

  40. Malik SN, Farmer PM, Hajdu SI, Rosenthal A (1996) Mesenchymal chondrosarcoma of the cerebellum. Ann Clin Lab Sci 26:496–500

    CAS  PubMed  Google Scholar 

  41. Marquardt G, Yahya H, Hermann E, Seifert V (2004) Direct transnasal approach for pituitary surgery. Neurosurg Rev 27:83–88

    Article  PubMed  Google Scholar 

  42. Meyers SP, Hirsch WL Jr, Curtin HD, Barnes L, Sekhar LN, Sen C (1992) Chondrosarcomas of the skull base: MR imaging features. Radiology 184:103–108

    CAS  PubMed  Google Scholar 

  43. Muthukumar N, Kondziolka D, Lunsford LD, Flickinger JC (1998) Stereotactic radiosurgery for chordoma and chondrosarcoma: further experiences. Int J Radiat Oncol Biol Phys 41:387–392

    Article  CAS  PubMed  Google Scholar 

  44. Naka T, Boltze C, Kuester D, Samii A, Herold C, Ostertag H et al (2005) Histogenesis of intralesional fibrous septum in chordoma. Pathol Res Pract 201:443–447

    Article  PubMed  Google Scholar 

  45. Naka T, Boltze C, Kuester D, Samii A, Herold C, Ostertag H et al (2005) Intralesional fibrous septum in chordoma: a clinicopathologic and immunohistochemical study of 122 lesions. Am J Clin Pathol 124:288–294

    Article  PubMed  Google Scholar 

  46. Naka T, Boltze C, Samii A, Herold C, Ostertag H, Iwamoto Y et al (2003) Skull base and nonskull base chordomas: clinicopathologic and immunohistochemical study with special reference to nuclear pleomorphism and proliferative ability. Cancer 98:1934–1941

    Article  PubMed  Google Scholar 

  47. Noel G, Habrand JL, Mammar H, Pontvert D, Haie-Meder C, Hasboun D et al (2001) Combination of photon and proton radiation therapy for chordomas and chondrosarcomas of the skull base: the Centre de Protontherapie D’Orsay experience. Int J Radiat Oncol Biol Phys 51:392–398

    Article  CAS  PubMed  Google Scholar 

  48. Pamir MN, Ozduman K (2006) Analysis of radiological features relative to histopathology in 42 skull-base chordomas and chondrosarcomas. Eur J Radiol 58:461–470

    Article  PubMed  Google Scholar 

  49. Patel NP, Mhatre AN, Lalwani AK (2004) Molecular pathogenesis of skull base tumors. Otol Neurotol 25:636–643

    Article  PubMed  Google Scholar 

  50. Podlaha J, Pavlica F (1928) Das bösartige sacrococcygeale Chordom. Ein neuer Fall; pathologisch-anatomische Studie. Virchow Arch 267:363–378

    Article  Google Scholar 

  51. Rosenberg AE, Nielsen GP, Keel SB, Renard LG, Fitzek MM, Munzenrider JE, Liebsch NJ (1999) Chondrosarcoma of the base of the skull: a clinicopathologic study of 200 cases with emphasis on its distinction from chordoma. Am J Surg Pathol 23:1370–1378

    Article  CAS  PubMed  Google Scholar 

  52. Samii M, Emirati M (1988) The combined supra-infratentorial pre-sigmoid sinus avenue to the petro-clival region. Surgical technique and clinical applications. Acta Neurochir (Wien) 95:6–12

    Article  CAS  Google Scholar 

  53. Samii M, Draf W (1989) Surgery of space-occupying lesions of the lateral middle skull base. In: Samii M, Draf W (eds) Surgery of the skull base – an interdisciplinary approach. Springer, Berlin/Heidelberg, pp 296–358

    Chapter  Google Scholar 

  54. Samii M, Draf W (1989) Surgery of the clivus. In: Samii M, Draf W (eds) Surgery of the skull base – an interdisciplinary approach. Springer, Berlin/Heidelberg, pp 432–460

    Chapter  Google Scholar 

  55. Samii M, Klekamp J, Carvalho G (1996) Surgical results for meningiomas of the craniocervical junction. Neurosurgery 39:1086–1095

    Article  CAS  PubMed  Google Scholar 

  56. Samii M, Tatagiba M, Carvalho GA (1999) Resection of large petroclival meningiomas by the simple retrosigmoid route. J Clin Neurosci 6:27–30

    Article  PubMed  Google Scholar 

  57. Samii M, Tatagiba M, Carvalho GA (2000) Retrosigmoid intradural suprameatal approach to Meckel’s cave and the middle fossa: surgical technique and outcome. J Neurosurg 92:235–241

    Article  CAS  PubMed  Google Scholar 

  58. Samii M, Tatagiba M (2004) Basic principles of skull base surgery. In: Winn HR (ed) Youmans neurological surgery. Saunders, Philadelphia, pp 909–929

    Google Scholar 

  59. Schulz-Ertner D, Haberer T, Jakel O, Thilmann C, Kramer M, Enghardt W et al (2002) Radiotherapy for chordomas and low-grade chondrosarcomas of the skull base with carbon ions. Int J Radiat Oncol Biol Phys 53:36–42

    Article  PubMed  Google Scholar 

  60. Schulz-Ertner D, Nikoghosyan A, Hof H, Didinger B, Combs SE, Jakel O et al (2007) Carbon ion radiotherapy of skull base chondrosarcomas. Int J Radiat Oncol Biol Phys 67:171–177

    Article  PubMed  Google Scholar 

  61. Schulz-Ertner D, Nikoghosyan A, Thilmann C, Haberer T, Jakel O, Karger C et al (2003) Carbon ion radiotherapy for chordomas and low-grade chondrosarcomas of the skull base. Results in 67 patients. Strahlenther Onkol 179:598–605

    Article  PubMed  Google Scholar 

  62. Schulz-Ertner D, Nikoghosyan A, Thilmann C, Haberer T, Jakel O, Karger C et al (2004) Results of carbon ion radiotherapy in 152 patients. Int J Radiat Oncol Biol Phys 58:631–640

    Article  PubMed  Google Scholar 

  63. Sekhar LN, Chanda A, Chandrasekar K, Wright DC (2004) Chordoma and chondrosarcomas. In: Winn HR (ed) Youmans neurological surgery. Saunders, Philadelphia, pp 1283–1294

    Google Scholar 

  64. Sekhar LN, Pranatartiharan R, Chanda A, Wright DC (2001) Chordomas and chondrosarcomas of the skull base: results and complications of surgical management. Neurosurg Focus 10:E2

    Article  CAS  PubMed  Google Scholar 

  65. Sekhar LN, Wright DC (1999) Resection of anterior, middle, and posterior cranial base tumors via the extended subfrontal approach. In: Sekhar LN, De Oliveira E (eds) Cranial microsurgery – approaches and techniques. Thieme, New York, pp 82–90

    Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  67. Tai PT, Craighead P, Bagdon F (1995) Optimization of radiotherapy for patients with cranial chordoma. A review of dose–response ratios for photon techniques. Cancer 75:749–756

    Article  CAS  PubMed  Google Scholar 

  68. Taniguchi M, Kohmura E (2012) Endoscopic endonasal removal of laterally extended clival chordoma using side-viewing scopes. Acta Neurochir (Wien) 154:627–632

    Article  Google Scholar 

  69. 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

    Article  PubMed  Google Scholar 

  70. Yassa M, Bahary JP, Bourguoin P, Belair M, Berthelet F, Bouthillier A (2005) Intra-parenchymal mesenchymal chondrosarcoma of the cerebellum: case report and review of the literature. J Neurooncol 74:329–331

    Article  PubMed  Google Scholar 

  71. Yeom KW, Lober RM, Mobley BC et al (2013) Diffusion-weighted MRI: distinction of skull base chordoma from chondrosarcoma. AJNR Am J Neuroradiol 34:1056–1061

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Marcos Tatagiba MD, PhD .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Tatagiba, M., Acioly, M.A. (2014). Chordomas and Chondrosarcomas. In: Ramina, R., de Aguiar, P., Tatagiba, M. (eds) Samii's Essentials in Neurosurgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54115-5_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-54115-5_18

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-54114-8

  • Online ISBN: 978-3-642-54115-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics