Chordoma Management: A Review of the Literature
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Published:
Feb 20, 2019
Keywords:
chordoma, recurrent chordoma, treatment of chordoma
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Abstract
Chordoma is an uncommon bone neoplasm. Despite being considered a low-grade or benign bone tumor because of its histology, it is aggressive and locally invasive. It has a poor prognosis because it has a high rate of recurrence and is likely to be malignant. Chordoma is usually recurrent. Although its histology is considered a low-grade tumor or benign tumor, its behaviour of recurrence leads to a poor prognosis and higher likelihood of malignant tumors occurring. Chordoma is believed to emerge from transformed remnants of notochord and appears to favor the axial bone. The most commonly affected parts of chordoma are at the sacrum, vertebra, and skull base. The present gold standard for management of chordoma is a wide surgical resection. This article highlights recent practices and future directions for treatment.
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1.
Lertboonyapan J, Pruksakorn D. Chordoma Management: A Review of the Literature. BKK Med J [Internet]. 2019 Feb. 20 [cited 2024 Nov. 22];15(1):107. Available from: https://he02.tci-thaijo.org/index.php/bkkmedj/article/view/222716
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Reviews Article
This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
References
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12. Horten BC, Montague SR. In vitro characteristics of asacrococcygeal chordoma maintained in tissue and organculture systems. Acta Neuropathol 1976;35(1):13-25.
13. Crapanzano JP, Ali SZ, Ginsberg MS, et al. Chordoma: acytologic study with histologic and radiologic correlation.Cancer 2001;93(1):40-51.
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16. Nibu Y, Jose-Edwards DS, Di Gregorio A. From notochordformation to hereditary chordoma: the many roles of Brachyury.Biomed Res Int 2013;2013:826435.
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18. Li M, Zhai Y, Bai J, et al. SNF5 as a prognostic factor in skullbase chordoma. J Neurooncol 2018;137(1):139-46.
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22. Catton C, O’Sullivan B, Bell R, et al. Chordoma: long-termfollow-up after radical photon irradiation. Radiother Oncol1996;41(1):67-72.
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24. York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma:40-year experience at a major cancer center. Neurosurgery1999;44(1):74-9; discussion 9-80.
25. Stener B, Gunterberg B. High amputation of the sacrum forextirpation of tumors. Principles and technique. Spine (PhilaPa 1976) 1978;3(4):351-66.
26. Hsieh PC, Xu R, Sciubba DM, et al. Long-term clinicaloutcomes following en bloc resections for sacral chordomasand chondrosarcomas: a series of twenty consecutive patients.Spine (Phila Pa 1976) 2009;34(20):2233-9.
27. Tzortzidis F, Elahi F, Wright D, et al. Patient outcome atlong-term follow-up after aggressive microsurgical resectionof cranial base chordomas. Neurosurgery 2006;59(2):230-7;discussion -7.
28. Fuchs B, Dickey ID, Yaszemski MJ, et al. Operativemanagement of sacral chordoma. J Bone Joint Surg Am2005;87(10):2211-6.
29. Samson IR, Springfield DS, Suit HD, et al. Operative treatmentof sacrococcygeal chordoma. A review of twenty-one cases.J Bone Joint Surg Am 1993;75(10):1476-84.
30. Stacchiotti S, Gronchi A, Fossati P, et al. Best practices forthe management of local-regional recurrent chordoma: aposition paper by the Chordoma Global Consensus Group.Ann Oncol 2017;28(6):1230-42.
31. Austin-Seymour M, Munzenrider JE, Goitein M, et al.Progress in low-LET heavy particle therapy: intracranial andparacranial tumors and uveal melanomas. Radiat Res Suppl1985;8:S219-26.
32. Suit HD, Goitein M, Munzenrider J, et al. Definitive radiationtherapy for chordoma and chondrosarcoma of base of skulland cervical spine. J Neurosurg 1982;56(3):377-85.
33. Austin-Seymour M, Munzenrider J, Linggood R, et al.Fractionated proton radiation therapy of cranial and intracranialtumors. Am J Clin Oncol 1990;13(4):327-30.
34. Imai R, Kamada T, Tsuji H, et al. Carbon ion radiotherapy forunresectable sacral chordomas. Clin Cancer Res2004;10(17):5741-6.
35. Nishida Y, Kamada T, Imai R, et al. Clinical outcome of sacralchordoma with carbon ion radiotherapy compared withsurgery. Int J Radiat Oncol Biol Phys 2011;79(1):110-6.
36. Schneider C, Vyfhuis M, Morse E, et al. Dramatic Responseof a Large Sacral Chordoma to Intensity Modulated ProtonBeam Therapy. Cureus 2017;9(9):e1670.
37. Durante M, Loeffler JS. Charged particles in radiation oncology.Nat Rev Clin Oncol 2010;7(1):37-43.
38. Negri T, Casieri P, Miselli F, et al. Evidence for PDGFRA,PDGFRB and KIT deregulation in an NSCLC patient. Br JCancer 2007;96(1):180-1.
39. Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylatein chordoma. Cancer 2004;101(9):2086-97.
40. Stacchiotti S, Marrari A, Tamborini E, et al. Response toimatinib plus sirolimus in advanced chordoma. Ann Oncol2009;20(11):1886-94.
41. Rinner B, Froehlich EV, Buerger K, et al. Establishment anddetailed functional and molecular genetic characterisation ofa novel sacral chordoma cell line, MUG-Chor1. Int J Oncol2012;40(2):443-51.
42. Magnaghi P, Salom B, Cozzi L, et al. Afatinib Is a NewTherapeutic Approach in Chordoma with a Unique Ability toTarget EGFR
2. 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):1-11.
3. Sciubba DM, Chi JH, Rhines LD, et al. Chordoma of thespinal column. Neurosurg Clin N Am 2008;19(1):5-15.
4. Wold LE, Laws ER, Jr. Cranial chordomas in children andyoung adults. J Neurosurg 1983;59(6):1043-7.
5. Llauger J, Palmer J, Amores S, et al. Primary tumors of thesacrum: diagnostic imaging. AJR Am J Roentgenol2000;174(2):417-24.
6. Cheng EY, Ozerdemoglu RA, Transfeldt EE, et al. Lumbosacralchordoma. Prognostic factors and treatment. Spine (Phila Pa1976) 1999;24(16):1639-45.
7. Ailon T, Torabi R, Fisher CG, et al. Management of LocallyRecurrent Chordoma of the Mobile Spine and Sacrum: ASystematic Review. Spine (Phila Pa 1976) 2016;41 Suppl20:S193-S8.
8. Fourney DR, Gokaslan ZL. Current management of sacralchordoma. Neurosurg Focus 2003;15(2):E9.
9. Bergh P, Kindblom LG, Gunterberg B, et al. Prognostic factorsin chordoma of the sacrum and mobile spine: a study of 39patients. Cancer 2000;88(9):2122-34.
10. Bjornsson J, Wold LE, Ebersold MJ, et al. Chordoma of themobile spine. A clinicopathologic analysis of 40 patients.Cancer 1993;71(3):735-40.
11. Chambers PW, Schwinn CP. Chordoma. A clinicopathologicstudy of metastasis. Am J Clin Pathol 1979;72(5):765-76.
12. Horten BC, Montague SR. In vitro characteristics of asacrococcygeal chordoma maintained in tissue and organculture systems. Acta Neuropathol 1976;35(1):13-25.
13. Crapanzano JP, Ali SZ, Ginsberg MS, et al. Chordoma: acytologic study with histologic and radiologic correlation.Cancer 2001;93(1):40-51.
14. Holland PW, Koschorz B, Holland LZ, et al. Conservation ofBrachyury (T) genes in amphioxus and vertebrates:developmental and evolutionary implications. Development1995;121(12):4283-91.
15. Yang XR, Ng D, Alcorta DA, et al. T (brachyury) geneduplication confers major susceptibility to familial chordoma.Nat Genet 2009;41(11):1176-8.
16. Nibu Y, Jose-Edwards DS, Di Gregorio A. From notochordformation to hereditary chordoma: the many roles of Brachyury.Biomed Res Int 2013;2013:826435.
17. Presneau N, Shalaby A, Ye H, et al. Role of the transcriptionfactor T (brachyury) in the pathogenesis of sporadicchordoma: a genetic and functional-based study. J Pathol2011;223(3):327-35.
18. Li M, Zhai Y, Bai J, et al. SNF5 as a prognostic factor in skullbase chordoma. J Neurooncol 2018;137(1):139-46.
19. Zhai Y, Bai J, Wang S, et al. Analysis of clinical factors andPDGFR-beta in predicting prognosis of patients with clivalchordoma. J Neurosurg 2018:1-9.
20. Freeman JL, DeMonte F, Al-Holou W, et al. Impact of earlyaccess to multidisciplinary care on treatment outcomes inpatients with skull base chordoma. Acta Neurochir (Wien)2018;160(4):731-40.
21. Pallini R, Maira G, Pierconti F, et al. Chordoma of the skullbase: predictors of tumor recurrence. J Neurosurg2003;98(4):812-22.
22. Catton C, O’Sullivan B, Bell R, et al. Chordoma: long-termfollow-up after radical photon irradiation. Radiother Oncol1996;41(1):67-72.
23. Boriani S, Chevalley F, Weinstein JN, et al. Chordoma of thespine above the sacrum. Treatment and outcome in 21 cases.Spine (Phila Pa 1976) 1996;21(13):1569-77.
24. York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma:40-year experience at a major cancer center. Neurosurgery1999;44(1):74-9; discussion 9-80.
25. Stener B, Gunterberg B. High amputation of the sacrum forextirpation of tumors. Principles and technique. Spine (PhilaPa 1976) 1978;3(4):351-66.
26. Hsieh PC, Xu R, Sciubba DM, et al. Long-term clinicaloutcomes following en bloc resections for sacral chordomasand chondrosarcomas: a series of twenty consecutive patients.Spine (Phila Pa 1976) 2009;34(20):2233-9.
27. Tzortzidis F, Elahi F, Wright D, et al. Patient outcome atlong-term follow-up after aggressive microsurgical resectionof cranial base chordomas. Neurosurgery 2006;59(2):230-7;discussion -7.
28. Fuchs B, Dickey ID, Yaszemski MJ, et al. Operativemanagement of sacral chordoma. J Bone Joint Surg Am2005;87(10):2211-6.
29. Samson IR, Springfield DS, Suit HD, et al. Operative treatmentof sacrococcygeal chordoma. A review of twenty-one cases.J Bone Joint Surg Am 1993;75(10):1476-84.
30. Stacchiotti S, Gronchi A, Fossati P, et al. Best practices forthe management of local-regional recurrent chordoma: aposition paper by the Chordoma Global Consensus Group.Ann Oncol 2017;28(6):1230-42.
31. Austin-Seymour M, Munzenrider JE, Goitein M, et al.Progress in low-LET heavy particle therapy: intracranial andparacranial tumors and uveal melanomas. Radiat Res Suppl1985;8:S219-26.
32. Suit HD, Goitein M, Munzenrider J, et al. Definitive radiationtherapy for chordoma and chondrosarcoma of base of skulland cervical spine. J Neurosurg 1982;56(3):377-85.
33. Austin-Seymour M, Munzenrider J, Linggood R, et al.Fractionated proton radiation therapy of cranial and intracranialtumors. Am J Clin Oncol 1990;13(4):327-30.
34. Imai R, Kamada T, Tsuji H, et al. Carbon ion radiotherapy forunresectable sacral chordomas. Clin Cancer Res2004;10(17):5741-6.
35. Nishida Y, Kamada T, Imai R, et al. Clinical outcome of sacralchordoma with carbon ion radiotherapy compared withsurgery. Int J Radiat Oncol Biol Phys 2011;79(1):110-6.
36. Schneider C, Vyfhuis M, Morse E, et al. Dramatic Responseof a Large Sacral Chordoma to Intensity Modulated ProtonBeam Therapy. Cureus 2017;9(9):e1670.
37. Durante M, Loeffler JS. Charged particles in radiation oncology.Nat Rev Clin Oncol 2010;7(1):37-43.
38. Negri T, Casieri P, Miselli F, et al. Evidence for PDGFRA,PDGFRB and KIT deregulation in an NSCLC patient. Br JCancer 2007;96(1):180-1.
39. Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylatein chordoma. Cancer 2004;101(9):2086-97.
40. Stacchiotti S, Marrari A, Tamborini E, et al. Response toimatinib plus sirolimus in advanced chordoma. Ann Oncol2009;20(11):1886-94.
41. Rinner B, Froehlich EV, Buerger K, et al. Establishment anddetailed functional and molecular genetic characterisation ofa novel sacral chordoma cell line, MUG-Chor1. Int J Oncol2012;40(2):443-51.
42. Magnaghi P, Salom B, Cozzi L, et al. Afatinib Is a NewTherapeutic Approach in Chordoma with a Unique Ability toTarget EGFR