Abstract
Chordoma is a relatively rare, slow-growing, primary bone tumor with an overall incidence of approximately one per million population and accounts for 1–4% of all malignant bone lesions [1, 2]. Although rare, it represents the most frequent primary malignant bone tumor affecting the sacrum [3]. It has a slowly aggressive and locally invasive behavior, and it is considered a low-grade malignant neoplasm. In fact, it is poorly sensitive to conventional radiotherapy and chemotherapy. Surgical resection of sacral chordoma remains the standard for local disease control, even if it is associated with significant morbidity and repercussions for patient’s quality of life due to the close relationship with relevant neurovascular structures [4]. An increasing number of novel (radio)surgical and pharmacological strategies are currently being investigated [5–7] and may have a role in addressing microscopic disease.
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References
Healey JH, Lane JM. Chordoma: a critical review of diagnosis and treatment. Orthop Clin North Am. 1989;20:417–26.
Smoll NR, Gautschi OP, Radovanovic I, Schaller K, Weber DC. Incidence and relative survival of chordomas: the standardized mortality ratio and the impact of chordomas on a population. Cancer. 2013;119:2029–37.
Fabbri N, Ruggieri P. Chordoma. In: Picci P, et al., editors. Atlas of musculoskeletal tumors and tumor like lesions. Switzerland: Springer; 2014. p. 233–8.
Casali PG, Stacchiotti S, Sangalli C, Olmi P, Gronchi A. Chordoma. Curr Opin Oncol. 2007;19:367–70.
Garofalo F, di Summa PG, Christoforidis D, Pracht M, Laudato P, Cherix S, Bouchaab H, Raffoul W, Demartines N, Matter M. Multidisciplinary approach of lumbo-sacral chordoma: from oncological treatment to reconstructive surgery. J Surg Oncol. 2015;112(5):544–54.
Amichetti M, Cianchetti M, Amelio D, Enrici RM, Minniti G. Proton therapy in chordoma of the base of the skull: a systematic review. Neurosurg Rev. 2009;32:403–16.
Staab A, Rutz HP, Ares C, et al. Spot-scanning-based proton therapy for extracranial chordoma. Int J Radiat Oncol Biol Phys. 2011;81:e489–96.
Luschka H. Die Altersveränderungen der Zwischenwirbelknorpel. Virchows Arch A Pathol Anat Histol. 1856;9:311–27.
Kyriakos M, Totty WG, Lenke LG. Giant vertebral notochordal rest: a lesion distinct from chordoma: discussion of an evolving concept. Am J Surg Pathol. 2003;27:396–406.
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.
Yakkioui Y, van Overbeeke JJ, Santegoeds R, van Engeland M, Temel Y. Chordoma: the entity. Biochim Biophys Acta. 2014;1846(2):655–69.
O’Rahilly R, Meyer DB. The timing and sequence of events in the development of the human vertebral column during the embryonic period proper. Anat Embryol. 1979;157(2):167–76.
Schoenwolf GC, Larsen WJ. Third week: becomming trilaminar and establishing body axis, Larsen’s human embryology. Philadelphia: Elsevier/Churchill Livingstone; 2009.
Bumcrot DA, McMahon AP. Somite differentiation. Curr Biol. 1995;5:612–4.
Bami M, Mavrogenis AF, Angelini A, Milonaki M, Mitsiokapa E, Stamoulis D, Soucacos PN. Bone morphogenetic protein signaling in musculoskeletal cancer. J Cancer Res Clin Oncol. 2016;142(10):2061–72.
Liem Jr KF, Jessell TM, Briscoe J. Regulation of the neural patterning activity of sonic hedgehog by secreted BMP inhibitors expressed by notochord and somites. Development. 2000;127:4855–66.
Stemple DL. Structure and function of the notochord: an essential organ for chordate development. Development. 2005;132:2503–12.
Dietrich S, Schubert FR, Gruss P. Altered Pax gene expression in murine notochord mutants: the notochord is required to initiate and maintain ventral identity in the somite. Mech Dev. 1993;44:189–207.
Pourquie O, Coltey M, Teillet MA, Ordahl C, Le Douarin NM. Control of dorsoventral patterning of somitic derivatives by notochord and floor plate. Proc Natl Acad Sci U S A. 1993;90:5242–6.
Alvares LE, Lours C, El-Hanfy A, Dietrich S. Microsurgical manipulation of the notochord. Methods Mol Biol. 2008;461:289–303.
Claudio DS. Grafting of somites. Methods Mol Biol. 2008;461:277–87.
Resende TP, Ferreira M, Teillet MA, Tavares AT, Andrade RP, Palmeirim I. Sonic hedgehog in temporal control of somite formation. Proc Natl Acad Sci U S A. 2010;107(29):12907–12.
Gilbert SF. Developmental biology. Sunderland: Sinauer Associates; 2010.
Moore KL, Persaud TVN, Torchia MG. The developing human: clinically oriented embryology. Philadelphia: Saunders/Elsevier; 2008.
Bogduk N. Embryology and development, Clinical anatomy of the lumbar spine and sacrum. Edinburgh: Elsevier/Churchill Livingstone; 2005. p. 149–63.
Oka Y, Sato Y, Tsuda H, Hanaoka K, Hirai Y, Takahashi Y. Epimorphin acts extracellularly to promote cell sorting and aggregation during the condensation of vertebral cartilage. Dev Biol. 2006;291(1):25–37.
Rodrigo I, Hill RE, Balling R, Münsterberg A, Imai K. Pax1 and Pax9 activate Bapx1 to induce chondrogenic differentiation in the sclerotome. Development. 2003;130(3):473–82.
Hunter CJ, Matyas JR, Duncan NA. The notochordal cell in the nucleus pulposus: a review in the context of tissue engineering. Tissue Eng. 2003;9(4):667–77.
Yamaguchi T, Suzuki S, Ishiiwa H, Ueda Y. Intraosseous benign notochordal cell tumours: overlooked precursors of classic chordomas? Histopathology. 2004;44(6):597–602.
Virchow R. Untersuchungen uber die Entwickelung des Schadelgrundes im gesunden und krankhaften Zustande, und uber den Einfluss derselben auf Schadelform, Gesichtsbildung und Gehirnbau. Berl: G. Rimer; 1857. p. 47.
Ribbert H. Uber die Eccondrosis physaliphora spheno-occipitalis. Zentralbl Allg Pathol Anat. 1894;5:457.
Salisbury JR. The pathology of the human notochord. J Pathol. 1993;171:253–5.
Vujovic S, Henderson S, Presneau N, et al. Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol. 2006;209:157–65.
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;18:96–9.
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;237:3953–8.
Yang XR, Ng D, Alcorta DA, et al. T (brachyury) gene duplication confers major susceptibility to familial chordoma. Nat Genet. 2009;41:1176–8.
Nibu Y, José-Edwards DS, Di Gregorio A. From notochord formation to hereditary chordoma: the many roles of Brachyury. Biomed Res Int. 2013;2013:826435.
Gulluoglu S, Turksoy O, Kuskucu A, Ture U, Bayrak OF. The molecular aspects of chordoma. Neurosurg Rev. 2016;39(2):185–96.
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.
Jemal A, Siegel R, Ward E, et al. Cancer statistics, 2007. CA Cancer J Clin. 2007;57:43–66.
Chugh R, Tawbi H, Lucas DR, et al. Chordoma: the nonsarcoma primary bone tumor. Oncologist. 2007;12:1344–50.
Papagelopoulos PJ, Mavrogenis AF, Galanis EC, Savvidou OD, Boscainos PJ, Katonis PG, Sim FH. Chordoma of the spine: clinicopathological features, diagnosis, and treatment. Orthopedics. 2004;27(12):1256–63.
Anson KM, Byrne PO, Robertson ID, Gullan RW, Montgomery AC. Radical excision of sacrococcygeal tumours. Br J Surg. 1994;81:460–1.
Smith J, Ludwig RL, Marcove RC. Sacrococcygeal chordoma. A clinic radiological study of 60 patients. Skeletal Radiol. 1987;16:37–44.
Kayani B, Hanna SA, Sewell MD, Saifuddin A, Molloy S, Briggs TW. A review of the surgical management of sacral chordoma. Eur J Surg Oncol. 2014;40(11):1412–20.
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;88:2122–34.
Kaiser TE, Pritchard DJ, Unni KK. Clinicopathologic study of sacrococcygeal chordoma. Cancer. 1984;53:2574–8.
Gray SW, Singhabhandhu B, Smith RA, Skandalakis JE. Sacrococcygeal chordoma: report of a case and review of the literature. Surgery. 1975;78:573–82.
Fourney DR, Gokaslan ZL. Current management of sacral chordoma. Neurosurg Focus. 2003;15:9.
Ozaki T, Hillmann A, Winkelmann W. Surgical treatment of sacrococcygeal chordoma. J Surg Oncol. 1997;64(4):274–9.
Dahan H, Arrive L, Wendum D, et al. Retrorectal developmental cysts in adults: Clinical and radiologic-histopathologic review, differential diagnosis, and treatment. Radiographics. 2001;21:575–84.
Williams LS, Rojiani AM, Quisling RG, et al. Retrorectal cyst-hamartomas and sacral dysplasia: MR appearance. AJNR Am J Neuroradiol. 1998;19:1043–5.
Lim KE, Hsu WC, Wang CR. Tailgut cyst with malignancy: MR imaging findings. AJR Am J Roentgenol. 1998;170:1488–90.
Bjornsson J, Wold LE, Ebersold MJ, Laws ER. Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer. 1993;71:735–40.
Llauger J, Palmer J, Amores S, et al. Primary tumors of the sacrum: diagnostic imaging. AJR Am J Roentgenol. 2000;174:417–24.
Rossleigh MA, Smith J, Yeh SD. Scintigraphic features of primary sacral tumors. J Nucl Med. 1986;27:627–30.
Crapanzano JP, Ali SZ, Ginsberg MS, Zakowski MF. Chordoma: a cytologic study with histologic and radiologic correlation. Cancer. 2001;93:40–51.
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;72:2943–9.
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;21:1461–9.
Paidakakos NA, Rovlias A, Rokas E, Theodoropoulos S, Katafygiotis P. Primary clear cell chondrosarcoma of the spine: a case report of a rare entity and a review of the literature. Case Rep Oncol Med. 2012;2012:693137.
Jeffrey PB, Biava CG, Davis RL. Chondroid chordoma. A hyalinized chordoma without cartilaginous differentiation. Am J Clin Pathol. 1995;103(3):271–9.
Saito A, Hasegawa T, Shimoda T, Toda G, Hirohashi S, Tajima G, et al. Dedifferentiated chordoma: a case report. Jpn J Clin Oncol. 1998;28:766–71.
Kim SC, Cho W, Chang UK, Youn SM. Two cases of dedifferentiated chordoma in the sacrum. Korean J Spine. 2015;12(3):230–4.
Meis JM, Raymond AK, Evans HL, Charles RE, Giraldo AA. “De-differentiated” chordoma: a clinicopathologic and immunohistochemical study of three cases. Am J Surg Pathol. 1987;11:516–25.
Bisceglia M, D’Angelo VA, Guglielmi G, Dor DB, Pasguinelli. De-differentiated chordoma of the thoracic spine with rhabdomyosarcomatous differentiations: report of a case and review of the literature. Ann Diagn Pathol 2007;11:262–273.
Kishikawa H, Tanaka K. Chordoma: report of an autopsy case with fibrosarcoma. Acta Pathol Jpn. 1974;24:299–308.
Morimitsu Y, Aoki T, Yokoyama K, Hashimoto H. Sarcomatoid chordoma: chordoma with a massive spindle cell component. Skeletal Radiol. 2000;29:721–5.
Fukuda T, Aihara T, Ban S, Nakajima T, Machinami R. Sacrococcygeal chordoma with malignant spindle cell component: a report of two autopsy cases with a review of the literature. Acta Pathol Jpn. 1992;42:448–53.
Fuchs B, Dickey ID, Yaszemski MJ, Inwards CY, Sim FH. Operative management of sacral chordoma. J Bone Joint Surg Am. 2005;87:2211–6.
Ruggieri P, Angelini A, Ussia G, Montalti M, Mercuri M. Surgical margins and local control in resection of sacral chordomas. Clin Orthop Relat Res. 2010;468:2939–47.
Angelini A, Pala E, Calabrò T, Maraldi M, Ruggieri P. Prognostic factors in surgical resection of sacral chordoma. J Surg Oncol. 2015;112(4):344–51.
Jawad MU, Scully SP. Surgery significantly improves survival in patients with chordoma. Spine. 2010;35:117–23.
Romeo S, Hogendoorn PC. Brachyury and chordoma: the chondroid-chordoid dilemma resolved? J Pathol. 2006;209:143–6.
Park JB, Lee CK, Koh JS, Lee JK, Park EY, Riew KD. Overexpressions of nerve growth factor and its tropomyosin-related kinase A receptor on chordoma cells. Spine. 2007;32(18):1969–73.
Hulen CA, Temple T, Fox WP, Sama AA, Green BA, Eismont FJ. Oncologic and functional outcome following sacrectomy for sacral chordoma. J Bone Joint Surg Am. 2006;88-A:1532–9.
Randall RL, Bruckner J, Lloyd C, Pohlman TH, Conrad EU. Sacral resection and reconstruction for tumors and tumor-like conditions. Orthopaedics. 2005;28:307–13.
Stener B, Gunterberg B. High amputation of the sacrum for extirpation of tumors. Principles and technique. Spine. 1978;3:351–66.
Varga PP, Szövérfi Z, Fisher CG, Boriani S, Gokaslan ZL, Dekutoski MB, Chou D, Quraishi NA, Reynolds JJ, Luzzati A, Williams R, Fehlings MG, Germscheid NM, Lazary A, Rhines LD. Surgical treatment of sacral chordoma: prognostic variables for local recurrence and overall survival. Eur Spine J. 2015;24(5):1092–101.
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;34:2233–9.
Atalar H, Selek H, Yildiz Y, et al. Management of sacrococcygeal chordomas. Int Orthop. 2006;30:514–8.
Devin C, Chong PY, Holt GE, et al. Level-adjusted perioperative risk of sacral amputations. J Surg Oncol. 2006;94:203–11.
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;75:1476–84.
Angelini A, Ruggieri P. A new surgical technique (modified Osaka technique) of sacral resection by posterior-only approach: description and preliminary results. Spine. 2013;38(3):E185–92.
Osaka S, Yoshida Y, Ryu J. Longitudinal osteotomy of lateral sacrum for malignant iliac tumor using modified threadwire saw. J Surg Oncol. 2007;95(3):258–60.
Gunterberg B, Kewenter J, Petersen I, Stener B. Anorectal function after major resections of the sacrum with bilateral or unilateral sacrifice of sacral nerves. Br J Surg. 1976;63(7):546–54.
Gunterberg B, Norlén L, Stener B, Sundin T. Neurourologic evaluation after resection of the sacrum. Invest Urol. 1975;13(3):183–8.
Gunterberg B, Petersen I. Sexual function after major resection of the sacrum with bilateral or unilateral sacrifice of sacral nerves. Fertil Steril. 1976;27:1146–53.
Zoccali C, Skoch J, Patel AS, Walter CM, Maykowski P, Baaj AA. Residual neurological function after sacral root resection during en-bloc sacrectomy: a systematic review. Eur Spine J. 2016;25. [Epub ahead of print].
Angelini A, Drago G, Trovarelli G, Calabrò T, Ruggieri P. Infection after surgical resection for pelvic bone tumors: an analysis of 270 patients from one institution. Clin Orthop Relat Res. 2014;472(1):349–59.
Chen KW, Yang HL, Lu J, et al. Risk factors for postoperative wound infections of sacral chordoma after surgical excision. J Spinal Disord Tech. 2011;24:230–4.
Pennicooke B, Laufer I, Sahgal A, Varga PP, Gokaslan ZL, Bilsky MH, Yamada YJ. Safety and local control of radiation therapy for chordoma of the spine and sacrum: a systematic review. Spine. 2016;41(Suppl 20):S186–92.
Rich TA, Schiller A, Suit HD, Mankin HJ. Clinical and pathologic review of 48 cases of chordoma. Cancer. 1985;56:182–7.
Catton C, O’Sullivan B, Bell R, et al. Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol. 1996;41:67–72.
Cummings BJ, Hodson DI, Bush RS. Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys. 1983;9:633–42.
Rotondo RL, Folkert W, Liebsch NJ, et al. High-dose proton-based radiation therapy in the management of spine chordomas: outcomes and clinicopathological prognostic factors. J Neurosurg Spine. 2015;23:788–97.
Zabel-du Bois A, Nikoghosyan A, Schwahofer A, et al. Intensity modulated radiotherapy in the management of sacral chordoma in primary versus recurrent disease. Radiother Oncol. 2010;97:408–12.
Hug EB, Fitzek MM, Liebsch NJ, et al. Locally challenging osteo- and chondrogenic tumors of the axial skeleton: results of combined proton and photon radiation therapy using three-dimensional treatment planning. Int J Radiat Oncol Biol Phys. 1995;31:467–76.
Yamada Y, Laufer I, Cox BW, et al. Preliminary results of high-dose single-fraction radiotherapy for the management of chordomas of the spine and sacrum. Neurosurgery. 2013;73:673–80.
Brown JM, Koong AC. High-dose single-fraction radiotherapy: exploiting a new biology? Int J Radiat Oncol Biol Phys. 2008;71:324–5.
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;56:377–85.
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;8:219–26.
Tobias CA, Blakely EA, Alpen EL, et al. Molecular and cellular radiobiology of heavy ions. Int J Radiat Oncol Biol Phys. 1982;8:2109–20.
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.
McDonald MW, Linton OR, Shah MV. Proton therapy for reirradiation of progressive or recurrent chordoma. Int J Radiat Oncol Biol Phys. 2013;87:1107–14.
Indelicato DJ, Rotondo RL, Begosh-Mayne D, et al. A prospective outcomes study of proton therapy for chordomas and chondrosarcomas of the spine. Int J Radiat Oncol Biol Phys. 2016;95:297–303.
Scholz M, Kraft G. Track structure and the calculation of biological effects of heavy charged particles. Adv Space Res. 1996;18:5–14.
Imai R, Kamada T, Tsuji H, et al. Carbon ion radiotherapy for unresectable sacral chordomas. Clin Cancer Res. 2004;10:5741–6.
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;79:110–6.
Imai R, Kamada T, Sugahara S, et al. Carbon ion radiotherapy for sacral chordoma. Br J Radiol. 2011;84(Spec. No. 1):S48–54.
Uhl M, Welzel T, Jensen A, Ellerbrock M, Haberer T, Jäkel O, Herfarth K, Debus J. Carbon ion beam treatment in patients with primary and recurrent sacrococcygeal chordoma. Strahlenther Onkol. 2015;191(7):597–603.
Lundkvist J, Ekman M, Ericsson SR, Jönsson B, Glimelius B. Proton therapy of cancer: potential clinical advantages and cost-effectiveness. Acta Oncol. 2005;44:850–61.
Durante M, Loeffler JS. Charged particles in radiation oncology. Nat Rev Clin Oncol. 2010;7:37–43.
Mohan R, Bortfeld T. Proton therapy: clinical gains through current and future treatment programs. Front Radiat Ther Oncol. 2011;43:440–64.
Goitein M, Cox JD. Should randomized clinical trials be required for proton radiotherapy? J Clin Oncol. 2008;26:175–6.
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;65:1514–21.
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;86:148–53.
Patt JC. CORR Insights(®): sacral insufficiency fractures are common after high-dose radiation for sacral chordomas treated with or without surgery. Clin Orthop Relat Res. 2016;474(3):773–5.
Negri T, Casieri P, Miselli F, et al. Evidence for PDGFRA, PDGFRB and KIT deregulation in an NSCLC patient. Br J Cancer. 2007;96:180–1.
Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylate in chordoma. Cancer. 2004;101:2086–97.
Stacchiotti S, Marrari A, Tamborini E, et al. Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol. 2009;20:1886–94.
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;27:3154–60.
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.
Stacchiotti S, Longhi A, Ferraresi V, et al. Phase II study of imatinib in advanced chordoma. J Clin Oncol. 2012;30:914–20.
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;131:707–11.
Hof H, Welzel T, Debus J. Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma. Onkologie. 2006;29:572–4.
Singhal N, Kotasek D, Parnis FX. Response to erlotinib in a patient with treatment refractory chordoma. Anticancer Drugs. 2009;20:953–5.
Stacchiotti S, Tamborini E, Lo Vullo S, et al. Phase II study on lapatinib in advanced EGFR-positive chordoma. Ann Oncol. 2013;24:1931–6.
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;8:2597–605.
Yang C, Hornicek FJ, Wood KB, et al. Blockage of Stat3 with CDDO-Me inhibits tumor cell growth in chordoma. Spine. 2010;35:1668–75.
Thieblemont C, Biron P, Rocher F, Bouhour D, Bobin JY, Gérard JP, et al. Prognostic factors in chordoma: role of postoperative radiotherapy. Eur J Cancer. 1995;31A:2255–9.
McGirt MJ, Gokaslan ZL, Chaichana KL. Preoperative grading scale to predict survival in patients undergoing resection of malignant primary osseous spinal neoplasms. Spine J. 2011;11(3):190–6. doi:10.1016/j.spinee.2011.01.013.
Ozger A, Eralp L, Sungur M, Atalar AC. Surgical management of sacral chordoma. Acta Orthop Belg. 2010;76:243–53.
Lee J, Bhatia N, Hoang B, Ziogas A, Zell J. Analysis of prognostic factors for patients with chordoma with use of the California Cancer Registry. J Bone Joint Surg Am. 2012;94:356–63.
Hobusch GM, Bodner F, Walzer S, Marculescu R, Funovics PT, Sulzbacher I, Windhager R, Panotopoulos J. C-reactive protein as a prognostic factor in patients with chordoma of lumbar spine and sacrum—a single center pilot study. World J Surg Oncol. 2016;14:111.
Hanna SA, Aston WJ, Briggs TW, Cannon SR, Saifuddin A. Sacral chordoma: can local recurrence after sacrectomy be predicted? Clin Orthop Relat Res. 2008;466(9):2217–23.
Boriani S, Bandiera S, Biagini R, Bacchini P, Boriani L, Cappuccio M, Chevalley F, Gasbarrini A, Picci P, Weinstein JN. Chordoma of the mobile spine: fifty years of experience. Spine. 2006;31(4):493–503.
Stacchiotti S, Casali PG, Lo Vullo S, Mariani L, Palassini E, Mercuri M, 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.
von Witzleben A, Goerttler LT, Lennerz J, Weissinger S, Kornmann M, Mayer-Steinacker R, von Baer A, Schultheiss M, Möller P, Barth TF. In chordoma, metastasis, recurrences, Ki-67 index, and a matrix-poor phenotype are associated with patients’ shorter overall survival. Eur Spine J. 2016;25:4016–24.
Ridenour 3rd RV, Ahrens WA, Folpe AL, Miller DV. Clinical and histopathologic features of chordomas in children and young adults. Pediatr Dev Pathol. 2010;13(1):9–17.
Cheng EY, Ozerdemoglu RA, Transfeldt EE, Thompson Jr RC. Lumbosacral chordoma. Prognostic factors and treatment. Spine. 1999;24:1639–45.
Chen KW, Yang HL, Lu J, Liu JY, Chen XQ. Prognostic factors of sacral chordoma after surgical therapy: a study of 36 patients. Spinal Cord. 2009;48:166–71.
Ishii K, Chiba K, Watanabe M, Yabe H, Fujimura Y, Toyama Y. Local recurrence after S2-3 sacrectomy in sacral chordoma. Report of four cases. J Neurosurg. 2002;97(1 Suppl):98–101.
Dorfman HD, Czerniak B. Bone cancers. Cancer. 1995;75:203–10.
Mukherjee D, Chaichana KL, Parker SL, Gokaslan ZL, McGirt MJ. Association of surgical resection and survival in patients with malignant primary osseous spinal neoplasms from the surveillance, epidemiology, and end results (SEER) database. Eur Spine J. 2012;22:1375.
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Angelini, A., Ruggieri, P. (2017). Chordoma of the Sacrum. In: Ruggieri, P., Angelini, A., Vanel, D., Picci, P. (eds) Tumors of the Sacrum. Springer, Cham. https://doi.org/10.1007/978-3-319-51202-0_15
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