Chordoma: Current concepts, management, and future directions

The Lancet Oncology

Volumn 13, Issue 2, 2012, Pages

  Walcott, Brian P ^a   Nahed, Brian V ^a   Mohyeldin, Ahmed ^b   Coumans, Jean Valery ^a   Kahle, Kristopher T ^a   Ferreira, Manuel J ^c  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b THE OHIO STATE UNIVERSITY COLLEGE OF MEDICINE   (United States)

^c UNIVERSITY OF WASHINGTON   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ALKYLATING AGENT; ANTHRACYCLINE; CAMPTOTHECIN; CD135 ANTIGEN; CETUXIMAB; CISPLATIN; EPITHELIAL MEMBRANE ANTIGEN; GEFITINIB; IMATINIB; MUCIN 1; PLATELET DERIVED GROWTH FACTOR A; PLATELET DERIVED GROWTH FACTOR B; PROTEIN INHIBITOR; PROTEIN S 100; STAT PROTEIN INHIBITOR; STEM CELL FACTOR RECEPTOR; SUNITINIB; TRANSCRIPTION FACTOR; TRANSCRIPTION FACTOR BRACHYURY; UNCLASSIFIED DRUG; VASCULOTROPIN RECEPTOR 1; VASCULOTROPIN RECEPTOR 2; VASCULOTROPIN RECEPTOR 3;

BRACHYURY GENE; BRAIN HEMORRHAGE; CANCER INCIDENCE; CANCER RADIOTHERAPY; CARCINOGENESIS; CELL PROLIFERATION; CHORDOMA; CLIVAL CHORDOMA; COMPUTER ASSISTED TOMOGRAPHY; CRANIAL NERVE PARALYSIS; CYTOREDUCTIVE SURGERY; DRUG ACTIVITY; EN BLOCK EXCISION; ENDOSCOPIC SURGERY; EPISTAXIS; HADRON; HUMAN; IMMUNOREACTIVITY; INTENSITY MODULATED RADIATION THERAPY; ION THERAPY; NUCLEAR MAGNETIC RESONANCE IMAGING; PALLIATIVE THERAPY; PHOTON THERAPY; POPULATION RESEARCH; PRIORITY JOURNAL; PROGNOSIS; RECURRENT DISEASE; REOPERATION; REVIEW; SACRUM; SENSITIVITY AND SPECIFICITY; SPINE; SPINE SURGERY; SURVIVAL; SYMPTOMATOLOGY; TREATMENT DURATION; TUMOR GENE;

BONE NEOPLASMS; CHORDOMA; CLINICAL TRIALS, PHASE I AS TOPIC; GENE EXPRESSION REGULATION, NEOPLASTIC; HUMANS; PROGNOSIS; RARE DISEASES; RECEPTORS, PLATELET-DERIVED GROWTH FACTOR; SPINE; TREATMENT OUTCOME;

EID: 84856434969     PISSN: 14702045     EISSN: 14745488     Source Type: Journal    
DOI: 10.1016/S1470-2045(11)70337-0     Document Type: Review

References (99)

1. Healey JH, Lane JM Chordoma: a critical review of diagnosis and treatment. Orthop Clin North Am 1989, 20:417-426.
2. Bergh P, Kindblom LG, Gunterberg B, Remotti F, Ryd W, Meis-Kindblom JM Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients. Cancer 2000, 88:2122-2134.
3. Schwab JH, Boland PJ, Agaram NP, et al. Chordoma and chondrosarcoma gene profile: implications for immunotherapy. Cancer Immunol Immunother 2009, 58:339-349.
4. 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.
5. Wold LE, Laws ER Cranial chordomas in children and young adults. J Neurosurg 1983, 59:1043-1047.
6. Cheng EY, Ozerdemoglu RA, Transfeldt EE, Thompson RC Lumbosacral chordoma: prognostic factors and treatment. Spine 1999, 24:1639-1645.
7. Virchow RL Untersuchungen ueber die Entwicklung des Schaedelgrundes 1857, G Rimer, Berlin.
8. Horten BC, Montague SR In vitro characteristics of a sacrococcygeal chordoma maintained in tissue and organ culture systems. Acta Neuropathol 1976, 35:13-25.
9. Ribbert H Uber die Ecchondosis physaliphora sphenooccipitalis. Centralbl Allg Pathol Anat 1894, 5:457-461.
10. Salisbury JR, Deverell MH, Cookson MJ, Whimster WF Three-dimensional reconstruction of human embryonic notochords: clue to the pathogenesis of chordoma. J Pathol 1993, 171:59-62.
11. Salisbury JR The pathology of the human notochord. J Pathol 1993, 171:253-255.
12. Vujovic S, Henderson S, Presneau N, et al. Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol 2006, 209:157-165.
13. Shen J, Li C-D, Yang H-L, et al. Classic chordoma coexisting with benign notochordal cell rest demonstrating different immunohistological expression patterns of brachyury and galectin-3. J Clin Neurosci 2011, 18:96-99.
14. Choi K-S, 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, 237:3953-3958.
15. Yang XR, Ng D, Alcorta DA, et al. T (brachyury) gene duplication confers major susceptibility to familial chordoma. Nat Genet 2009, 41:1176-1178.
16. Henderson SR, Guiliano D, Presneau N, et al. A molecular map of mesenchymal tumors. Genome Biol 2005, 6:76.
17. Fernando RI, Litzinger M, Trono P, Hamilton DH, Schlom J, Palena C The T-box transcription factor Brachyury promotes epithelial-mesenchymal transition in human tumor cells. J Clin Invest 2010, 120:533-544.
18. Stark AM, Mehdorn HM Images in clinical medicine. Chondroid clival chordoma. N Engl J Med 2003, 349:10.
19. Kitai R, Yoshida K, Kubota T, et al. Clival chordoma manifesting as nasal bleeding: a case report. Neuroradiology 2005, 47:368-371.
20. Levi AD, Kucharczyk W, Lang AP, Schutz H Clival chordoma presenting with acute brain stem hemorrhage. Can J Neurol Sci 1991, 18:515-518.
21. Kaiser TE, Pritchard DJ, Unni KK Clinicopathologic study of sacrococcygeal chordoma. Cancer 1984, 53:2574-2578.
22. Gray SW, Singhabhandhu B, Smith RA, Skandalakis JE Sacrococcygeal chordoma: report of a case and review of the literature. Surgery 1975, 78:573-582.
23. Fourney DR, Gokaslan ZL Current management of sacral chordoma. Neurosurg Focus 2003, 15:9.
24. Boriani S, Chevalley F, Weinstein JN, et al. Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine 1996, 21:1569-1577.
25. Bjornsson J, Wold LE, Ebersold MJ, Laws ER Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer 1993, 71:735-740.
26. Disler DG, Miklic D Imaging findings in tumors of the sacrum. AJR Am J Roentgenol 1999, 173:1699-1706.
27. Llauger J, Palmer J, Amores S, Bagué S, Camins A Primary tumors of the sacrum: diagnostic imaging. AJR Am J Roentgenol 2000, 174:417-424.
28. Rossleigh MA, Smith J, Yeh SD Scintigraphic features of primary sacral tumors. J Nucl Med 1986, 27:627-630.
29. Chambers PW, Schwinn CP Chordoma: a clinicopathologic study of metastasis. Am J Clin Pathol 1979, 72:765-776.
30. York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery 1999, 44:74-79.
31. Azzarelli A, Quagliuolo V, Cerasoli S, et al. Chordoma: natural history and treatment results in 33 cases. J Surg Oncol 1988, 37:185-191.
32. Oakley GJ, Fuhrer K, Seethala RR Brachyury, SOX-9, and podoplanin, new markers in the skull base chordoma vs chondrosarcoma differential: a tissue microarray-based comparative analysis. Mod Pathol 2008, 21:1461-1469.
33. Arnautović KI, Al-Mefty O Surgical seeding of chordomas. Neurosurg Focus 2001, 10:7.
34. Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH Chordoma: the nonsarcoma primary bone tumor. Oncologist 2007, 12:1344-1350.
35. Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF Chordoma: a cytologic study with histologic and radiologic correlation. Cancer 2001, 93:40-51.
36. Mitchell A, Scheithauer BW, Unni KK, Forsyth PJ, Wold LE, McGivney DJ Chordoma and chondroid neoplasms of the spheno-occiput. An immunohistochemical study of 41 cases with prognostic and nosologic implications. Cancer 1993, 72:2943-2949.
37. Abenoza P, Sibley RK Chordoma: an immunohistologic study. Hum Pathol 1986, 17:744-747.
38. Sangoi AR, Karamchandani J, Lane B, et al. Specificity of brachyury in the distinction of chordoma from clear cell renal cell carcinoma and germ cell tumors: a study of 305 cases. Mod Pathol 2011, 24:425-429.
39. Stener B, Gunterberg B High amputation of the sacrum for extirpation of tumors. Principles and technique. Spine 1978, 3:351-366.
40. Hsieh PC, Xu R, Sciubba DM, et al. Long-term clinical outcomes following en bloc resections for sacral chordomas and chondrosarcomas: a series of twenty consecutive patients. Spine 2009, 34:2233-2239.
41. 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, 17:211-219.
42. Tzortzidis F, Elahi F, Wright D, Natarajan SK, Sekhar LN Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 2006, 59:230-237.
43. Fuchs B, Dickey ID, Yaszemski MJ, Inwards CY, Sim FH Operative management of sacral chordoma. J Bone Joint Surg Am 2005, 87:2211-2216.
44. Samson IR, Springfield DS, Suit HD, Mankin HJ Operative treatment of sacrococcygeal chordoma. A review of twenty-one cases. J Bone Joint Surg Am 1993, 75:1476-1484.
45. Holzmann D, Reisch R, Krayenbühl N, Hug E, Bernays RL The transnasal transclival approach for clivus chordoma. Minim Invasive Neurosurg 2010, 53:211-217.
46. Singh H, Harrop J, Schiffmacher P, Rosen M, Evans J Ventral surgical approaches to craniovertebral junction chordomas. Neurosurgery 2010, 66(suppl 3):96-103.
47. Potluri S, Jefferies SJ, Jena R, et al. Residual postoperative tumour volume predicts outcome after high-dose radiotherapy for chordoma and chondrosarcoma of the skull base and spine. Clin Oncol (R Coll Radiol) 2011, 23:199-208.
48. Dehdashti AR, Karabatsou K, Ganna A, Witterick I, Gentili F Expanded endoscopic endonasal approach for treatment of clival chordomas: early results in 12 patients. Neurosurgery 2008, 63:299-307.
49. Fraser JF, Nyquist GG, Moore N, Anand VK, Schwartz TH Endoscopic endonasal transclival resection of chordomas: operative technique, clinical outcome, and review of the literature. J Neurosurg 2010, 112:1061-1069.
50. Stüer C, Schramm J, Schaller C Skull base chordomas: management and results. Neurol Med Chir (Tokyo) 2006, 46:118-124.
51. Osaka S, Kodoh O, Sugita H, Osaka E, Yoshida Y, Ryu J Clinical significance of a wide excision policy for sacrococcygeal chordoma. J Cancer Res Clin Oncol 2006, 132:213-218.
52. Casali PG, Stacchiotti S, Sangalli C, Olmi P, Gronchi A Chordoma. Curr Opin Oncol 2007, 19:367-370.
53. Forsyth PA, Cascino TL, Shaw EG, et al. Intracranial chordomas: a clinicopathological and prognostic study of 51 cases. J Neurosurg 1993, 78:741-747.
54. Catton C, O'Sullivan B, Bell R, et al. Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol 1996, 41:67-72.
55. Cummings BJ, Hodson DI, Bush RS Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys 1983, 9:633-642.
56. Suit HD, Goitein M, Munzenrider J, et al. Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine. J Neurosurg 1982, 56:377-385.
57. Austin-Seymour M, Munzenrider JE, Goitein M, et al. Progress in low-LET heavy particle therapy: intracranial and paracranial tumors and uveal melanomas. Radiat Res Suppl 1985, 8:219-226.
58. Austin-Seymour M, Munzenrider J, Linggood R, et al. Fractionated proton radiation therapy of cranial and intracranial tumors. Am J Clin Oncol 1990, 13:327-330.
59. Tobias CA, Blakely EA, Alpen EL, et al. Molecular and cellular radiobiology of heavy ions. Int J Radiat Oncol Biol Phys 1982, 8:2109-2120.
60. Austin-Seymour M, Munzenrider J, Goitein M, et al. Fractionated proton radiation therapy of chordoma and low-grade chondrosarcoma of the base of the skull. J Neurosurg 1989, 70:13-17.
61. Benk V, Liebsch NJ, Munzenrider JE, Efird J, McManus P, Suit H Base of skull and cervical spine chordomas in children treated by high-dose irradiation. Int J Radiat Oncol Biol Phys 1995, 31:577-581.
62. Berson AM, Castro JR, Petti P, et al. Charged particle irradiation of chordoma and chondrosarcoma of the base of skull and cervical spine: the Lawrence Berkeley Laboratory experience. Int J Radiat Oncol Biol Phys 1988, 15:559-565.
63. Hug EB, Loredo LN, Slater JD, et al. Proton radiation therapy for chordomas and chondrosarcomas of the skull base. J Neurosurg 1999, 91:432-439.
64. Noël G, Feuvret L, Calugaru V, et al. 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 2005, 44:700-708.
65. Imai R, Kamada T, Tsuji H, et al. Carbon ion radiotherapy for unresectable sacral chordomas. Clin Cancer Res 2004, 10:5741-5746.
66. Nishida Y, Kamada T, Imai R, et al. Clinical outcome of sacral chordoma with carbon ion radiotherapy compared with surgery. Int J Radiat Oncol Biol Phys 2011, 79:110-116.
67. Hasegawa T, Ishii D, Kida Y, Yoshimoto M, Koike J, Iizuka H Gamma Knife surgery for skull base chordomas and chondrosarcomas. J Neurosurg 2007, 107:752-757.
68. Kano H, Iqbal FO, Sheehan J, et al. Stereotactic radiosurgery for chordoma: a report from the North American Gamma Knife Consortium. Neurosurgery 2011, 68:379-389.
69. Marucci L, Niemierko A, Liebsch NJ, Aboubaker F, Liu MCC, Munzenrider JE Spinal cord tolerance to high-dose fractionated 3D conformal proton-photon irradiation as evaluated by equivalent uniform dose and dose volume histogram analysis. Int J Radiat Oncol Biol Phys 2004, 59:551-555.
70. Terahara A, Niemierko A, Goitein M, et al. Analysis of the relationship between tumor dose inhomogeneity and local control in patients with skull base chordoma. Int J Radiat Oncol Biol Phys 1999, 45:351-358.
71. Goitein M, Cox JD Should randomized clinical trials be required for proton radiotherapy?. J Clin Oncol 2008, 26:175-176.
72. 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 versus recurrent tumor. Int J Radiat Oncol Biol Phys 2006, 65:1514-1521.
73. Lundkvist J, Ekman M, Ericsson SR, Jönsson B, Glimelius B Proton therapy of cancer: potential clinical advantages and cost-effectiveness. Acta Oncol 2005, 44:850-861.
74. Levin WP, Kooy H, Loeffler JS, DeLaney TF Proton beam therapy. Br J Cancer 2005, 93:849-854.
75. Durante M, Loeffler JS Charged particles in radiation oncology. Nat Rev Clin Oncol 2010, 7:37-43.
76. Rich TA, Schiller A, Suit HD, Mankin HJ Clinical and pathologic review of 48 cases of chordoma. Cancer 1985, 56:182-187.
77. Suit H, Kooy H, Trofimov A, et al. Should positive phase III clinical trial data be required before proton beam therapy is more widely adopted? No. Radiother Oncol 2008, 86:148-153.
78. Mohan R, Bortfeld T Proton therapy: clinical gains through current and future treatment programs. Front Radiat Ther Oncol 2011, 43:440-464.
79. Jensen AD, Nikoghosyan A, Ellerbrock M, Ecker S, Debus J, Münter MW Re-irradiation with scanned charged particle beams in recurrent tumours of the head and neck: acute toxicity and feasibility. Radiother Oncol 2011, published online June 1. 10.1016/j.radonc.2011.05.017.
80. Heylen S, Michielsen J, Mahieu G, Somville J Recurrent chordoma of the L2 vertebra: a case report. Acta Orthop Belg 2010, 76:416-419.
81. Matsumoto M, Watanabe K, Ishii K, et al. Complicated surgical resection of malignant tumors in the upper cervical spine after failed ion-beam radiation therapy. Spine 2010, 35:505-509.
82. Ito E, Saito K, Okada T, Nagatani T, Nagasaka T Long-term control of clival chordoma with initial aggressive surgical resection and gamma knife radiosurgery for recurrence. Acta Neurochir (Wien) 2010, 152:57-67.
83. Tamaki M, Aoyagi M, Kuroiwa T, Yamamoto M, Kishimoto S, Ohno K Clinical course and autopsy findings of a patient with clival chordoma who underwent multiple surgeries and radiation during a 10-year period. Skull Base 2007, 17:331-340.
84. Yang C, Hornicek FJ, Wood KB, et al. Characterization and analysis of human chordoma cell lines. Spine 2010, 35:1257-1264.
85. 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, 23:3597-3604.
86. Fleming GF, Heimann PS, Stephens JK, et al. Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer 1993, 72:714-718.
87. Negri T, Casieri P, Miselli F, et al. Evidence for PDGFRA, PDGFRB and KIT deregulation in an NSCLC patient. Br J Cancer 2007, 96:180-181.
88. Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylate in chordoma. Cancer 2004, 101:2086-2097.
89. Stacchiotti S, Marrari A, Tamborini E, et al. Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol 2009, 20:1886-1894.
90. George S, Merriam P, Maki RG, et al. Multicenter phase II trial of sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas. J Clin Oncol 2009, 27:3154-3160.
91. Weinberger PM, Yu Z, Kowalski D, et al. Differential expression of epidermal growth factor receptor, c-Met, and HER2/neu in chordoma compared with 17 other malignancies. Arch Otolaryngol Head Neck Surg 2005, 131:707-711.
92. Hof H, Welzel T, Debus J Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma. Onkologie 2006, 29:572-574.
93. Singhal N, Kotasek D, Parnis FX Response to erlotinib in a patient with treatment refractory chordoma. Anticancer Drugs 2009, 20:953-955.
94. Yang C, Schwab JH, Schoenfeld AJ, et al. A novel target for treatment of chordoma: signal transducers and activators of transcription 3. Mol Cancer Ther 2009, 8:2597-2605.
95. Yang C, Hornicek FJ, Wood KB, et al. Blockage of Stat3 with CDDO-Me inhibits tumor cell growth in chordoma. Spine 2010, 35:1668-1675.
96. Fagundes MA, Hug EB, Liebsch NJ, Daly W, Efird J, Munzenrider JE Radiation therapy for chordomas of the base of skull and cervical spine: patterns of failure and outcome after relapse. Int J Radiat Oncol Biol Phys 1995, 33:579-584.
97. Stacchiotti S, Casali PG Systemic therapy options for unresectable and metastatic chordomas. Curr Oncol Rep 2011, 13:323-330.
98. Nikoghosyan AV, Karapanagiotou-Schenkel I, Münter MW, Jensen AD, Combs SE, Debus J Randomised trial of proton vs carbon ion radiation therapy in patients with chordoma of the skull base, clinical phase III study HIT-1-Study. BMC Cancer 2010, 10:607.
99. Hsu W, Mohyeldin A, Shah SR, et al. Generation of chordoma cell line JHC7 and the identification of Brachyury as a novel molecular target. J Neurosurg 2011, 115:760-769.