Current Treatment Options in Oncology

, Volume 14, Issue 3, pp 442–453 | Cite as

Multidisciplinary Management of Recurrent Chordomas

  • Yoshiya YamadaEmail author
  • Mrinal Gounder
  • Ilya Laufer
Sarcoma (SH Okuno, Section Editor)

Opinion statement

The management of recurrent chordomas are clinically challenging because of its relentless nature. Local therapy, whether surgery or radiation, are important considerations since local progression of disease results in significant morbidity and locally aggressive treatment is often required. Stereotactic radiosurgery, shown to be very effective for radioresistant histologies, may be an important radiotherapeutic approach for recurrent tumors. Ultimately, the treatment of recurrent chordoma is palliative in intent, thus, enthusiasm for improving local control must be tempered against the possible impact of treatment on quality of life. Judicious use of radiotherapy and surgery can often provide meaningful palliation and local control of recurrences. Systemic treatment options, particularly with targeted molecules have great potential for chordomas in the recurrent setting, as the risk of disseminated disease is higher. The development of tools to help assess potential targets for drug therapy will be crucial. The incorporation of locally aggressive therapy and effective systemic therapy will be critical for the successful management of recurrent chordomas. At present, there is a paucity of published data regarding salvage therapy. Nonetheless, advances in surgical, medical, and radiation oncology are providing new avenues of research and potentially may have significant impact upon successful salvage treatment.


Chordomas Radiation Stereotactic radiosurgery Radioresistant histologies Treatment Surgery Therapy Defferentiated chordoma Defferentiated chordoma  Radiotherapy 


Conflicts of Interest

Yoshiya Yamada is a consultant to Varian Medical Systems.

Mrinal Gounder declares that he has no conflict of interest. Ilya Laufer is a consultant to SpineWave and DePuy.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. 1.
    McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control. 2001;12:1–11.PubMedCrossRefGoogle Scholar
  2. 2.
    Enneking WF. A system of staging musculoskeletal neoplasms. Clin Orthop Related Res. 1986;204:9–24.Google Scholar
  3. 3.
    Boriani S, Weinstein JN, Biagini R. Primary bone tumors of the spine. Terminology and surgical staging. Spine. 1997;22:1036–44.PubMedCrossRefGoogle Scholar
  4. 4.
    Boriani S, Bandiera S, Biagini R, et al. Chordoma of the mobile spine: fifty years of experience. Spine. 2006;31:493–503.PubMedCrossRefGoogle Scholar
  5. 5.
    Bergh P, Gunterberg B, Meis Kindblom JM, Kindblom LG. Prognostic factors and outcome of pelvic, sacral, and spinal chondrosarcomas: a center-based study of 69 cases. Cancer. 2001;91:1201–12.PubMedCrossRefGoogle Scholar
  6. 6.
    Fuchs B, Dickey ID, Yaszemski MJ, Inwards CY, Sim FH. Operative management of sacral chordoma. J Bone Joint Surg [Am]. 2005;87:2211–6.CrossRefGoogle Scholar
  7. 7.
    Ruggieri P, Angelini A, Ussia G, Montalti M, Mercuri M. Surgical margins and local control in resection of sacral chordomas. Clin Orthop Related Res. 2010;468:2939–47.CrossRefGoogle Scholar
  8. 8.
    Schwab J, Healey J, Rose P, Casas Ganem J, Boland P. The surgical management of sacral chordomas. Spine. 2009;34:2700–4.PubMedCrossRefGoogle Scholar
  9. 9.•
    Stacchiotti S, Casali PG, Lo Vullo S, et al. Chordoma of the mobile spine and sacrum: a retrospective analysis of a series of patients surgically treated at two referral centers. Ann Surg Oncol. 2010;17:211–9. Largest series of chordoma detailing surgical outcomes.PubMedCrossRefGoogle Scholar
  10. 10.
    Fourney DR, Rhines LD, Hentschel SJ, et al. En bloc resection of primary sacral tumors: classification of surgical approaches and outcome. J Neurosurg Spine. 2005;3:111–22.PubMedCrossRefGoogle Scholar
  11. 11.
    Rich TA, Schiller A, Suit HD, Mankin HJ. Clinical and pathologic review of 48 cases of chordoma. Cancer. 1985;56:182–7.PubMedCrossRefGoogle Scholar
  12. 12.
    York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery. 1999;44:74–9. discussion 79–80.PubMedCrossRefGoogle Scholar
  13. 13.
    Chou WC, Hung YS, Lu CH, Yeh KY, Sheu S, Liaw CC. De novo dedifferentiated chordoma of the sacrum: a case report and review of the literature. Chang Gung Med J. 2009;32:330–5.PubMedGoogle Scholar
  14. 14.
    Chugh R, Dunn R, Zalupski MM, et al. Phase II study of 9-nitro-camptothecin in patients with advanced chordoma or soft tissue sarcoma. J Clin Oncol. 2005;23:3597–604.PubMedCrossRefGoogle Scholar
  15. 15.
    Henderson SR, Guiliano D, Presneau N, et al. A molecular map of mesenchymal tumors. Genome Biol. 2005;6(9):R76.PubMedCrossRefGoogle Scholar
  16. 16.•
    Presneau N, Shalaby A, Ye H, et al. Role of the transcription factor T (brachyury) in the pathogenesis of sporadic chordoma: a genetic and functional-based study. J Pathol. 2011;223:327–35. Described the discovery of brachyury, a transcription factor critical in chordoma.PubMedCrossRefGoogle Scholar
  17. 17.
    Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylate in chordoma. Cancer. 2004;101:2086–97.PubMedCrossRefGoogle Scholar
  18. 18.•
    Tamborini E, Virdis E, Negri T, et al. Analysis of receptor tyrosine kinases (RTKs) and downstream pathways in chordomas. Neuro-Oncology. 2010;12:776–89. Showed in chordoma cell lines that inhibition of upstream RTK by imatinib induces a resistance pathway by upregulation of down-stream targets such as mTOR.PubMedCrossRefGoogle Scholar
  19. 19.
    Schwab J, Antonescu C, Boland P, et al. Combination of PI3K/mTOR inhibition demonstrates efficacy in human chordoma. Anticancer Res. 2009;29:1867–71.PubMedGoogle Scholar
  20. 20.
    Presneau N, Shalaby A, Idowu B, et al. Potential therapeutic targets for chordoma: PI3K/AKT/TSC1/TSC2/mTOR pathway. Br J Cancer. 2009;100:1406–14.PubMedCrossRefGoogle Scholar
  21. 21.
    Stacchiotti S, Marrari A, Tamborini E, et al. Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol. 2009;20:1886–94.PubMedCrossRefGoogle Scholar
  22. 22.
    Hof H, Welzel T, Debus J. Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma. Onkologie. 2006;29:572–4.PubMedCrossRefGoogle Scholar
  23. 23.
    Linden O, Stenberg L, Kjellen E. Regression of cervical spinal cord compression in a patient with chordoma following treatment with cetuximab and gefitinib. Acta Oncol. 2009;48:158–9.PubMedCrossRefGoogle Scholar
  24. 24.
    Stacchiotti S, Tamborini E, Lo Vullo S, et al. Phase II study on lapatinib in advanced EGFR-positive chordoma. Ann Oncol. 2013;24:1931–1936.Google Scholar
  25. 25.
    Choi KS, Cohn MJ, Harfe BD. Identification of nucleus pulposus precursor cells and notochordal remnants in the mouse: implications for disk degeneration and chordoma formation. Dev Dyn. 2008;237:3953–8.PubMedCrossRefGoogle Scholar
  26. 26.
    Casali PG, Stacchiotti S, Grosso F, Messina A, Crippa E, Tamborin E, et al. Adding cisplatin to imatinib re-establishes tumor response following secondary resistance to IM in advanced chordoma. J Clin Oncol. 2007;25:10038.Google Scholar
  27. 27.
    Cummings BJ, Hodson DI, Bush RS. Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys. 1983;9:633–42.PubMedCrossRefGoogle Scholar
  28. 28.
    Bjornsson J, Wold LE, Ebersold MJ, Laws ER. Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer. 1993;71:735–40.PubMedCrossRefGoogle Scholar
  29. 29.•
    DeLaney TF, Liebsch NJ, Pedlow FX, et al. Phase II study of high-dose photon/proton radiotherapy in the management of spine sarcomas. Int J Radiat Oncol Biol Phys. 2009;74:732–9. Prospective study of patients treated with radiotherapy as the primary modality of treamtent.PubMedCrossRefGoogle Scholar
  30. 30.
    Garcia-Barros M, Paris F, Cordon-Cardo C, et al. Tumor response to radiotherapy regulated by endothelial cell apoptosis. Science. 2003;300:1155–9.PubMedCrossRefGoogle Scholar
  31. 31.
    Damast S, Wright J, Bilsky M, et al. Impact of dose on local failure rates after image-guided reirradiation of recurrent paraspinal metastases. Int J Radiat Oncol Biol Phys. 2011;81(3):819–26.Google Scholar
  32. 32.
    Garg AK, Wang XS, Shiu AS, et al. Prospective evaluation of spinal reirradiation by using stereotactic body radiation therapy: The University of Texas MD Anderson Cancer Center experience. Cancer. 2011;117:3509–16.Google Scholar
  33. 33.
    Park L, Delaney TF, Liebsch NJ, et al. Sacral chordomas: impact of high-dose proton/photon-beam radiation therapy combined with or without surgery for primary vs recurrent tumor. Int J Radiat Oncol Biol Phys. 2006;65:1514–21.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of Radiation Oncology, Medical Oncology, Neurosurgery, Memorial Sloan Kettering Cancer CenterNew YorkUSA
  2. 2.Department of Medical Oncology, Memorial Sloan Kettering Cancer CenterNew YorkUSA
  3. 3.Department of Neurosurgery, Memorial Sloan Kettering Cancer CenterNew YorkUSA

Personalised recommendations