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Chordoma

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Bone Tumors
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Abstract

Chordomas are slow-growing, locally destructive, rare primary malignant bone tumors located in the neural axis [1, 2]. Once the diagnosis is made (with a combination of clinical, imagenological and histological aspects), the tumor has to be staged in order to provide the best treatment option. These tumors are challenging to treat, with many questions left open about their optimum treatment. Wide surgical resection with a 1 cm tumor-free margin remains the standard of care for achieving curative resection. Other complementary therapies described and in development are adjuvant radiotherapy, proton beam therapy and newer chemotherapy agents such as imatinib.The most important prognostic factor in these patients is to perform a resection with adequate margins, thus achieving oncological survival of up to 20 years. Recurrences have a worse prognosis and continue to be a greater challenge in the treatment of this type of bone tumor.

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References

  1. Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012;13(2):e69–76.

    Article  Google Scholar 

  2. Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH. Chordoma: the nonsarcoma primary bone tumor. Oncologist. 2007;12(11):1344–50.

    Article  Google Scholar 

  3. Fourney DR, Gokaslan ZL. Current management of sacral chordoma. Neurosurg Focus. 2003;15:E9.

    Article  Google Scholar 

  4. Maclean FM, Soo MY, Ng T. Chordoma: radiological-pathological correlation. Australas Radiol. 2005;49:261–8.

    Article  CAS  Google Scholar 

  5. Llauger J, Palmer J, Amores S, et al. Primary tumors of the sacrum: diagnostic imaging. AJR Am J Roentgenol. 2000;174:417–24.

    Article  CAS  Google Scholar 

  6. Yamaguchi T, Suzuki S, Ishiiwa H, et al. Benign notochordal cell tumors: a comparative histological study of benign notochordal cell tumors, classic chordomas, and notochordal vestigious of fetal intervertebral disks. Am J Surg Pathol. 2004;28:756–61.

    Article  Google Scholar 

  7. Kyriakos M. Benign notochordal lesions of the axial skeleton: A review and current appraisal. Skeletal Radiol. 2011;40(9):1141–52.

    Article  Google Scholar 

  8. Amer HZ, Hameed M. Intraosseous benign notochordal cell tumor. Arch Pathol Lab Med. 2010;134(2):283–8.

    Article  Google Scholar 

  9. Fuchs B, Dickey I, Yaszemski M, Inwards C, Sim F. Operative management of sacral chordoma. J Bone Joint Surg Am. 2005;87:2211–6.

    PubMed  Google Scholar 

  10. Fleming GF, Heimann PS, Stephens JK, et al. Dedifferentiated chordoma: response to aggressive chemotherapy in two cases. Cancer. 1993;72:714–8.

    Article  CAS  Google Scholar 

  11. Sciubba D, Cheng J, Petteys R, et al. Chordoma of the sacrum and vertebral bodies. J Am Acad Orthop Surg. 2009;17:708–17.

    Article  Google Scholar 

  12. Osaka S, Kodoh O, Sugita H, et al. Clinical significance of a wide excision policy for sacrococcygeal chordoma. J Cancer Res Clin Oncol. 2006;132:213–8.

    Article  Google Scholar 

  13. Hulen CA, Temple HT, et al. Oncologic and functional outcome following sacrectomy for sacral chordoma. J Bone Joint Surg Am: 1532–1539.

    Google Scholar 

  14. DeLaney T, Liebsch N, Pedlow F, et al. Phase II study of high dose proton/photon radiotherapy in the management of spine sarcomas. Int J Radiation Oncology Viol Phys. 2009;74:732–9.

    Article  CAS  Google Scholar 

  15. Tamborini E, Miselli F, Negri T, et al. Molecular and biochemical analyses of platelet derived growth factor receptor (PDGFR) beta, PDGFRA, in KIT receptors in chordomas. Clin Cancer Res. 2006;12:6920–8.

    Article  CAS  Google Scholar 

  16. Sciubba DM, Chi JH, Rhines LD, Gokaslan ZL. Chordoma of the spinal column. Neurosurg Clin N Am. 2008;19:5–15.

    Article  Google Scholar 

  17. Casali PG, Stacchiotti S, Messina A, et al. Imatinib mesylate in 18 advanced chordoma patients. J Clin Oncol. 2005;23:9012.

    Article  Google Scholar 

  18. Schwab JH, Boland PJ, Agaram NP, et al. Chordoma and chondrosarcoma gene profile: Implications for immunotherapy. Cancer Immunol Immunother. 2009;58(3):339–49.

    Article  CAS  Google Scholar 

  19. Bergh P, Kindblom L, Gunterberg B, et al. Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer. 2000;88:2122–34.

    Article  CAS  Google Scholar 

  20. Hanna SA, Tirabosco R, Amin A, et al. Dedifferentiated chordoma: a report of four cases arising “de novo”. J Bone Joint Surg Am. 2008;90:652–6.

    Article  CAS  Google Scholar 

  21. McMaster ML, Goldstein AM, Bromley CM, et al. Chordoma: Incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control. 2001;12:1–11.

    Article  CAS  Google Scholar 

  22. Boriani S, Schevalley F, Weinstein JN, et al. Chordoma of the spine above the sacrum: treatment and outcome in 21 cases. Spine. 1996;21:1569–77.

    Article  CAS  Google Scholar 

  23. Boriani S, Bandiera S, Biagini R, et al. Chordoma of the mobile spine: 50 years of experience. Spine. 2006;31:493–503.

    Article  Google Scholar 

  24. Chambers PW, Schwinn CP. Chordoma: a clinicopathologic study of metastasis. Am J Clin Pathol. 1979;72(5):765–76.

    Article  CAS  Google Scholar 

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Correspondence to Franklin H. Sim .

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Sim, F.H. (2021). Chordoma. In: Paulos, J., Poitout, D.G. (eds) Bone Tumors. Springer, London. https://doi.org/10.1007/978-1-4471-7501-8_32

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  • DOI: https://doi.org/10.1007/978-1-4471-7501-8_32

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