SpringerLink
Log in

Chondrosarcoma of the Axial Skeleton

  • Chapter
  • First Online:
Chondrosarcoma

  • 499 Accesses

Abstract

Chondrosarcomas are rare primary bone malignancies that produce cartilage matrix, most commonly found in the appendicular skeleton. Less than 10% of cases occur in the axial skeleton with the majority found in the spinal column. Conventional chondrosarcoma comprises the overwhelming majority of axial chondrosarcoma. Plain radiographs, computed tomography, and magnetic resonance imaging are the mainstays of imaging, and biopsy is necessary to obtain tissue diagnosis. Chemotherapy and radiation therapy are largely ineffective for the majority of chondrosarcomas. Surgical resection is the preferred therapeutic modality for axial chondrosarcoma with en bloc resection being more effective at achieving tumor-free margins than marginal excision or intralesional curettage with decreased rates of local recurrence and improved survival. However, morbidity is significant given the close proximity to critical neurovascular structures and should be performed at a high-volume center with an experienced multidisciplinary team.

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

Access this chapter

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 64.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 84.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free ship** worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Giuffrida AY, Burgueno JE, Koniaris LG, Guiterrez JC, Duncan R, Scully SP. Chondrosarcoma in the United States (1973 to 2003): an analysis of 2890 cases from the SEER database. J Bone Joint Surg Am. 2009;91:1063–72.

    Article  PubMed  Google Scholar 

  2. Boriani S, De Iure F, Bandiera S, et al. Chondrosarcoma of the mobile spine: report on 22 cases. Spine (Phila Pa 1976). 2000;25(7):804–12.

    Article  CAS  Google Scholar 

  3. Kerr DA, Rosenberg AE. Pathology of chordoma and chondrosarcoma of the axial skeleton. In: Harsh GR, Vaz-Guimaraes F, editors. Chordomas and chondrosarcomas of the skull base and spine. 2nd ed. London: Academic Press; 2018. p. 11–21.

    Chapter  Google Scholar 

  4. Fisher CG, Versteeg AL, Dea N, et al. Surgical management of spinal chondrosarcomas. Spine (Phila Pa 1976). 2016;41(8):678–85.

    Article  Google Scholar 

  5. Korten AG, ter Berg HJ, Spincemaille GH, van der Laan RT, van de Wel AM. Intracranial chondrosarcoma: review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry. 1998;65(1):88–92.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Al-Mefty K, Pravdenkova S, Colli BO, Al-Mefty O, Gokden M. Chordoma and chondrosarcoma: similar, but quite different, skull base tumors. Cancer. 2007;110(11):2457–67.

    PubMed  Google Scholar 

  7. York JE, Berk RH, Fuller GN, et al. Chondrosarcoma of the spine: 1954-1997. J Neurosurg. 1999;90:73–8.

    CAS  PubMed  Google Scholar 

  8. Rosenberg AE, Nielsen GP, Keel SB, 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. 1999;23(11):1370–8.

    Article  CAS  PubMed  Google Scholar 

  9. Shives TC, McLeod RA, Unni KK, Schray MF. Chondrosarcoma of the spine. J Bone Joint Surg Am. 1989;71(8):1158–65.

    Article  CAS  PubMed  Google Scholar 

  10. Enneking WF. Staging of musculoskeletal neoplasms. In: Sundaresan N, Schmidek HH, Schiller AL, Rosenthal DI, editors. Tumors of the spine: diagnosis and clinical management. Philadelphia: W.B. Saunders; 1990. p. 22–33.

    Google Scholar 

  11. Boriani S, Weinstein JN, Biagini R. Primary bone tumors of the spine: terminology and surgical staging. Spine (Phila Pa 1976). 1997;22(9):1036–44.

    Article  CAS  Google Scholar 

  12. Chan P, Boriani S, Fourney DR, et al. An assessment of the reliability of the Enneking and Weinstein-Boriani-Biagini classifications for staging of primary spinal tumors by the Spine Oncology Study Group. Spine (Phila Pa 1976). 2009;34(4):384–91.

    Article  Google Scholar 

  13. Hirsh LF, Thanki A, Spector HB. Primary spinal chondrosarcoma with 18-year follow-up: case report and literature review. Neurosurgery. 1984;14(6):747–9.

    Article  CAS  PubMed  Google Scholar 

  14. World Health Organization. Cartilage tumors. In: Fletcher CDM, Unni KK, Mertens F, eds. World Health Organization classification of tumors. Pathology and genetics. Tumors of soft tissue and bone. IARC Press; 150 Cours Albert Thomas, 69372 Lyon CEDEX 08, France. 2002: 234–57.

    Google Scholar 

  15. Angelini A, Guerra G, Mavrogenis AF, Pala E, Picci P, Ruggieri P. Clinical outcome of central conventional chondrosarcoma. J Surg Oncol. 2012;106(8):929–37.

    Article  PubMed  Google Scholar 

  16. Doyle L. Sarcoma classification: an update based on the 2013 World Health Organization classification of tumors of soft tissue and bone. Cancer. 2014;120(12):1763–74.

    Article  PubMed  Google Scholar 

  17. Katonis P, Alpantaki K, Michail K, et al. Spinal chondrosarcoma: a review. Sarcoma. 2011;2011:378957.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Gitelis S, Bertoni F, Picci P, Campanacci M. Chondrosarcoma of bone. The experience of the Istituto Ortopedico Rissoli. J Bone Joint Surg Am. 1981;63(8):1248–57.

    Article  CAS  PubMed  Google Scholar 

  19. Zoccali C, Baldi J, Attali D, et al. Intralesional vs. extralesional procedures for low-grade central chondrosarcoma: a systematic review of the literature. Arch Orthop Trauma Surg. 2018;138(7):929–37.

    Article  PubMed  Google Scholar 

  20. Zhou Q, Yang C, Chen M, Li L. Detection of exostosis glycosyltransferase gene mutation in non-hereditary osteochondromas of the mandibular condyle. Mol Clin Oncol. 2016;5(3):295–9.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Gelderblom H, Hongendoorn PC, Dijkstra SD, et al. The clinical approach towards chondrosarcoma. Oncologist. 2008;13(3):320–9.

    Article  PubMed  Google Scholar 

  22. Arshi A, Sharim J, Park DY, et al. Chondrosarcoma of the osseous spine: an analysis of epidemiology, patient outcomes, and prognostic factors using the SEER Registry from 1973 to 2012. Spine (Phila Pa 1976). 2017;42(9):644–52.

    Article  Google Scholar 

  23. Hallor KH, Staaf J, Bovee JVMG, et al. Genomic profiling of chondrosarcoma: chromosomal patterns in central and peripheral tumors. Clin Cancer Res. 2009;15(8):2685–94.

    Article  CAS  PubMed  Google Scholar 

  24. de Andrera CE, Reijnders CM, Kroon HM, et al. Secondary peripheral chondrosarcoma evolving from osteochondroma as a result of outgrowth of cells with functional EXT. Oncogene. 2012;31:1095–104.

    Article  CAS  Google Scholar 

  25. Schrage YM, Lam S, Jochemsen AG, et al. Central chondrosarcoma progression is associated with pRb pathway alterations: CDK4 down-regulation and p16 overexpression inhibit cell growth in vitro. J Cell Mol Med. 2009;13(9A):2843–52.

    Article  CAS  PubMed  Google Scholar 

  26. Bovee JV, Hogendoorn PC, Wuner JS, Alman BA. Cartilage tumors and bone development: molecular pathology and possible therapeutic targets. Nat Rev Cancer. 2010;10(7):481–8.

    Article  CAS  PubMed  Google Scholar 

  27. Mechri M, Riahi H, Sboui I, Bouaziz M, Vanhoenacker F, Ladeb M. Imaging of malignant primitive tumors of the spine. J Belg Soc Radiol. 2018;102(1):56.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Liu G, Wu G, Ghimire P, et al. Primary spinal chondrosarcoma: radiologic manifestations with histopathological correlation in eight patients and literature review. Clin Imaging. 2013;37(1):124–33.

    Article  PubMed  Google Scholar 

  29. Nooij MA, Whelan J, Bramwell VH, et al. Doxorubicin and cisplatin chemotherapy in high-grade spindle cell sarcomas of the bone, other than osteosarcoma or malignant fibrous histiocytoma: a European Osteosarcoma Intergroup study. Eur J Cancer. 2005;41(2):225–30.

    Article  CAS  PubMed  Google Scholar 

  30. Frezza AM, Cesari M, Baumhoer D, et al. Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. Eur J Cancer. 2015;51:374–81.

    Article  PubMed  Google Scholar 

  31. Whelan JS, Davis LE. Osteosarcoma, chondrosarcoma, and chordoma. J Clin Oncol. 2017;36(2):188–93.

    Article  PubMed  Google Scholar 

  32. Hsu W, Kosztowski TA, Zaidi HA, et al. Multidisciplinary management of primary tumors of the vertebral column. Curr Treat Options in Oncol. 2009;10:107–25.

    Article  Google Scholar 

  33. Trieb K, Sulzbacher I, Kubista B. Recurrence rate and progression of chondrosarcoma is correlated with heat shock protein expression. Oncol Lett. 2016;11(1):521–4.

    Article  CAS  PubMed  Google Scholar 

  34. van Oosterwijk JG, Herpers B, Meijer D, et al. Restoration of chemosensitivity for doxorubicin and cisplatin in chondrosarcoma in vitro: BCL-2 family members cause chemoresistance. Ann Oncol. 2012;23(6):1617–26.

    Article  PubMed  Google Scholar 

  35. Thanindratarn P, Dean DC, Nelson SD, Hornicek FJ, Duan Z. Advances in immune checkpoint inhibitors for bone sarcoma therapy. J Bone Oncol. 2019;15:100221.

    Article  PubMed  PubMed Central  Google Scholar 

  36. de Amorim Bernstein K, Delaney T. Chordomas and chondrosarcomas – the role of radiation therapy. J Surg Oncol. 2016;114:564–9.

    Article  PubMed  Google Scholar 

  37. Holliday EB, Mitra HS, Somerson JS, et al. Postoperative proton therapy for chordomas and chondrosarcomas of the spine. Spine (Phila Pa 1976). 2015;40(8):544–9.

    Article  Google Scholar 

  38. Delaney TF, Liebsch NJ, Pedlow FX, et al. Long-term results of phase II study of high dose photon/proton radiotherapy in the management of spine chordomas, chondrosarcomas, and other sarcomas. J Surg Oncol. 2014;110:115–22.

    Article  PubMed  Google Scholar 

  39. Boriani S, Saravanja D, Yamada Y, Varga PP, Biagini R, Fisher CG. Challenges of local recurrence and cure in low grade malignant tumors of the spine. Spine (Phila Pa 1976). 2009;34(22S):S48–57.

    Article  Google Scholar 

  40. Weinstein JN, McLain RF. Primary tumors of the spine. Spine (Phila Pa 1976). 1987;12(9):843–51.

    Article  CAS  Google Scholar 

  41. Boriani S, Biagini R, De Iure F. En bloc resections of bone tumors of the thoracolumbar spine. A preliminary report on 29 patients. Spine (Phila Pa 1976). 1996;21(16):1927–31.

    Article  CAS  Google Scholar 

  42. Sundaresan N, Steinberger AA, Moore F, et al. Indications and results of combined anterior-posterior approaches for spine tumor surgery. J Neurosurg. 1996;85(3):438–46.

    Article  CAS  PubMed  Google Scholar 

  43. Fisher CG, Keynan O, Boyd MC, Dvorak MF. The surgical management of primary tumors of the spine: initial results of an ongoing prospective cohort study. Spine (Phila Pa 1976). 2005;30(16):1899–908.

    Article  Google Scholar 

  44. Cloyd JM, Acosta FL, Polley M, Ames CP. En bloc resection for primary and metastatic tumors of the spine: a systematic review of the literature. Neurosurgery. 2010;67:435–45.

    Article  PubMed  Google Scholar 

  45. Amendola L, Capuccio M, De Iure F, Bandiera S, Gasbarrini A, Boriani S. En bloc resections for primary spinal tumors in 20 years of experience: effectiveness and safety. Spine J. 2014;14(11):2608–17.

    Article  PubMed  Google Scholar 

  46. Sundaresan N, DiGiacinto GV, Krol G, Hughes JE. Spondylectomy for malignant tumors of the spine. J Clin Oncol. 1989;7(10):1485–91.

    Article  CAS  PubMed  Google Scholar 

  47. Stener B. Total spondylectomy in chondrosarcoma arising from the seventh thoracic vertebra. J Bone Joint Surg Br. 1971;53(2):288–95.

    Article  CAS  PubMed  Google Scholar 

  48. Stener B. Complete removal of vertebrae for extirpation of tumors. A 20-year experience. Clin Orthop Relat Res. 1989;245:72–82.

    Google Scholar 

  49. Fidler MW. Radical resection of vertebral body tumors. A surgical technique used in ten cases. J Bone Joint Surg Br. 1994;76(5):765–72.

    Article  CAS  PubMed  Google Scholar 

  50. Tomita K, Kawahara N, Baba HH, Tsuchiya H, Fujita T, Toribatake Y. Total en bloc spondylectomy. A new surgical technique for primary malignant vertebral tumors. Spine (Phila Pa 1976). 1997;22(3):324–33.

    Article  CAS  Google Scholar 

  51. Fourney DR, Abi-Said D, Rhines LD, et al. Simultaneous anterior-posterior approach to the thoracic and lumbar spine for the radical resection of tumors followed by reconstruction and stabilization. J Neurosurg. 2001;94(2 Suppl):232–44.

    CAS  PubMed  Google Scholar 

  52. Tomita K, Kawahara N, Murakami H, Demura S. Total en bloc spondylectomy for spinal tumors: improvement of the technique and its associated basic background. J Orthop Sci. 2006;11(1):3–12.

    Article  PubMed  PubMed Central  Google Scholar 

  53. Shah AA, Paulino Pereira NR, Pedlow FX, et al. Modified en bloc spondylectomy for tumors of the thoracic and lumbar spine: surgical technique and outcomes. J Bone Joint Surg Am. 2017;99(17):1476–84.

    Article  PubMed  Google Scholar 

  54. Boriani S, Bandiera S, Donthineni R, et al. Morbidity of en bloc resections in the spine. Eur Spine J. 2010;19(2):231–41.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. UCLA Department of Orthopaedic Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

    Akash A. Shah, Howard Young Park, Gideon Blumstein & Don Young Park

Authors
  1. Akash A. Shah
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Howard Young Park
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gideon Blumstein
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Don Young Park
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Don Young Park .

Editor information

Editors and Affiliations

  1. Department of Orthopaedic Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA

    Francis J. Hornicek

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Shah, A.A., Park, H.Y., Blumstein, G., Park, D.Y. (2021). Chondrosarcoma of the Axial Skeleton. In: Hornicek, F.J. (eds) Chondrosarcoma. Springer, Cham. https://doi.org/10.1007/978-3-030-74572-1_6

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-74572-1_6

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-74571-4

  • Online ISBN: 978-3-030-74572-1

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation