Neurosurgical Review

, Volume 31, Issue 1, pp 35–43 | Cite as

Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients

  • Young Hyun Cho
  • Jeong Hoon Kim
  • Shin Kwang Khang
  • Jung-Kyo Lee
  • Chang Jin Kim
Original Article


Chordomas and chondrosarcomas occur rarely in the skull base and have been often considered commonly with respect to the management. The aim of this study was to investigate the clinical results in each series of these tumors and analyze comparative outcome. Between 1991 and 2005, 30 consecutive patients with pathological diagnosis of chordoma (n = 19) or chondrosarcoma (n = 11) of the skull base were managed by multimodal treatment combining surgical resection with conventional photon radiotherapy and/or gamma knife radiosurgery. A retrospective analysis was conducted on these patients (aged 3 to 69 years; mean age of 37.3 years; 17 females and 13 males; mean follow-up of 56.1 months). Four deaths occurred among the patients with chordoma. The progression-free survival rate at 3 and 5 years was 61.5 and 40.0% in chordoma. In contrast, no mortalities were observed, and there was only one recurrence among the patients with chondrosarcoma. The progression-free survival rate at 3 and 5 years was 88.9 and 80.0% in chondrosarcoma. Among 15 survivors with chordoma, four patients were suffering from severe disability with progressive disease. On the other hand, most patients with chondrosarcoma harbored stable disease and less disabling symptoms except one instance of recurrence. As the biological behavior of chordoma is much more aggressive than that of chondrosarcoma in the skull base, one should make a distinction between these entities to plan an optimal treatment strategy.


Chordoma Chondrosarcoma Skull base Treatment outcome 



  1. 1.
    Borba LA, Al-Mefty O, Mrak RE, Suen J (1996) Cranial chordomas in children and adolescents. J Neurosurg 84:584–591PubMedGoogle Scholar
  2. 2.
    Brackmann DE, Teufert KB (2006) Chondrosarcoma of the skull base: long-term follow-up. Otol Neurotol 27:981–991PubMedCrossRefGoogle Scholar
  3. 3.
    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 41:67–72PubMedGoogle Scholar
  4. 4.
    Colli B, Al-Mefty O (2001) Chordomas of the craniocervical junction: follow-up review and prognostic factors. J Neurosurg 95:933–943PubMedGoogle Scholar
  5. 5.
    Crockard HA, Cheeseman A, Steel T, Revesz T, Holton JL, Plowman N, Singh A, Crossman J (2001) A multidisciplinary team approach to skull base chondrosarcomas. J Neurosurg 95:184–189PubMedCrossRefGoogle Scholar
  6. 6.
    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 95:175–183PubMedGoogle Scholar
  7. 7.
    Debus J, Schulz-Ertner D, Schad L, Essig M, Rhein B, Thillmann CO, Wannenmacher M (2000) Stereotactic fractionated radiotherapy for chordomas and chondrosarcomas of the skull base. Int J Radiat Oncol Biol Phys 47:591–596PubMedCrossRefGoogle Scholar
  8. 8.
    Feigl GC, Bundschuh O, Gharabaghi A, Safavi-Abassi S, El Shawarby A, Samii M, Horstmann GA (2005) Evaluation of a new concept for the management of skull base chordomas and chondrosarcomas. J Neurosurg 102(Suppl):165–170PubMedGoogle Scholar
  9. 9.
    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 36:887–896 (discussion 896–897)PubMedCrossRefGoogle Scholar
  10. 10.
    Hug EB, Loredo LN, Slater JD, DeVries A, Grove RI, Schaefer RA, Rosenberg AE, Slater JM (1999) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 91:432–439PubMedGoogle Scholar
  11. 11.
    Hug EB, Slater JD (2000) Proton radiation therapy for chordomas and chondrosarcomas of the skull base. Neurosurg Clin N Am 11:627–638PubMedGoogle Scholar
  12. 12.
    Krishnan S, Foote RL, Brown PD, Pollock BE, Link MJ, Garces YI (2005) Radiosurgery for cranial base chordomas and chondrosarcomas. Neurosurgery 56:777–783 (discussion 783–784)PubMedCrossRefGoogle Scholar
  13. 13.
    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 12:1–11PubMedCrossRefGoogle Scholar
  14. 14.
    Mendenhall WM, Mendenhall CM, Lewis SB, Villaret DB, Mendenhall NP (2005) Skull base chordoma. Head Neck 27:159–165PubMedCrossRefGoogle Scholar
  15. 15.
    Munzenrider JE, Liebsch NJ (1999) Proton therapy for tumors of the skull base. Strahlenther Onkol 175(Suppl 2):57–63PubMedGoogle Scholar
  16. 16.
    Muthukumar N, Kondziolka D, Lunsford LD, Flickinger JC (1998) Stereotactic radiosurgery for chordoma and chondrosarcoma: further experiences. Int J Radiat Oncol Biol Phys 41:387–392PubMedCrossRefGoogle Scholar
  17. 17.
    Noel G, Feuvret L, Calugaru V, Dhermain F, Mammar H, Haie-Meder C, Ponvert D, Hasboun D, Ferrand R, Nauraye C, Boisserie G, Beaudre A, Gaboriaud G, Mazal A, Habrand JL, Mazeron JJ (2005) 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 44:700–708PubMedCrossRefGoogle Scholar
  18. 18.
    Noel G, Feuvret L, Ferrand R, Boisserie G, Mazeron JJ, Habrand JL (2004) Radiotherapeutic factors in the management of cervical-basal chordomas and chondrosarcomas. Neurosurgery 55:1252–1260 (discussion 1260–1262)PubMedGoogle Scholar
  19. 19.
    Noel G, Habrand JL, Jauffret E, de Crevoisier R, Dederke S, Mammar H, Haie-Meder C, Pontvert D, Hasboun D, Ferrand R, Boisserie G, Beaudre A, Gaboriaud G, Guedea F, Petriz L, Mazeron JJ (2003) Radiation therapy for chordoma and chondrosarcoma of the skull base and the cervical spine. Prognostic factors and patterns of failure. Strahlenther Onkol 179:241–248PubMedCrossRefGoogle Scholar
  20. 20.
    Noel G, Habrand JL, Mammar H, Pontvert D, Haie-Meder C, Hasboun D, Moisson P, Ferrand R, Beaudre A, Boisserie G, Gaboriaud G, Mazal A, Kerody K, Schlienger M, Mazeron JJ (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 51:392–398PubMedCrossRefGoogle Scholar
  21. 21.
    Oghalai JS, Buxbaum JL, Jackler RK, McDermott MW (2005) Skull base chondrosarcoma originating from the petroclival junction. Otol Neurotol 26:1052–1060PubMedCrossRefGoogle Scholar
  22. 22.
    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 23:1370–1378PubMedCrossRefGoogle Scholar
  23. 23.
    Sen CN, Sekhar LN, Schramm VL, Janecka IP (1989) Chordoma and chondrosarcoma of the cranial base: an 8-year experience. Neurosurgery 25:931–940 (discussion 940–941)PubMedCrossRefGoogle Scholar
  24. 24.
    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 59:230–236 (discussion 237)PubMedCrossRefGoogle Scholar
  25. 25.
    Wanebo JE, Bristol RE, Porter RR, Coons SW, Spetzler RF (2006) Management of cranial base chondrosarcomas. Neurosurgery 58:249–254 (discussion 254–255)PubMedCrossRefGoogle Scholar
  26. 26.
    Weber AL, Liebsch NJ, Sanchez R, Sweriduk ST Jr (1994) Chordomas of the skull base. Radiologic and clinical evaluation. Neuroimaging Clin N Am 4:515–527PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • Young Hyun Cho
    • 1
  • Jeong Hoon Kim
    • 1
    • 3
  • Shin Kwang Khang
    • 2
  • Jung-Kyo Lee
    • 1
  • Chang Jin Kim
    • 1
  1. 1.Department of Neurological SurgeryAsan Medical Center, University of Ulsan College of MedicineSeoulSouth Korea
  2. 2.Department of PathologyAsan Medical Center, University of Ulsan College of MedicineSeoulSouth Korea
  3. 3.Department of NeurosurgeryAsan Medical CenterSeoulSouth Korea

Personalised recommendations