Abstract
Chordoma is a locally aggressive tumor that may develop in the spine, but in children more often arises from the base of the skull (clivus). It is a slowly growing mass that may cause bone destruction and extend into the intracranial compartment and sphenoid sinus causing pain and neurological symptoms. Rarely, metastasis to the lungs and lymph nodes is present. Complete tumor resection provides the best chances for long-term survival, but it is very difficult to obtain in children due to the tumor location. Therefore, high-dose proton therapy is administered. Prognosis has improved over the years arriving up to 80% 5-year survival. Targeted therapies with agents such as imatinib, gefitinib, and erlotinib are under investigation.
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References
Beccaria K, Sainte-Rose C, Zerah M et al (2015) Paediatric chordomas. Orphanet J Rare Dis 10(1):116
Borba LA, Al-Mefty O, Mrak RE, Suen J (1996) Cranial chordomas in children and adolescents. J Neurosurg 84(4):584–591
Dhall G, Traverso M, Finlay JL, Shane L, Gonzalez-Gomez I, Jubran R (2011) The role of chemotherapy in pediatric clival chordomas. J Neurooncol 103(3):657–662
Fung V, Calugaru V, Bolle S, Mammar H, Alapetite C, Maingon P, De Marzi L, Froelich S, Habrand JL, Dendale R, Noël G, Feuvret L (2018) Proton beam therapy for skull base chordomas in 106 patients: a dose adaptive radiation protocol. Radiother Oncol 128(2):198–202
Geoerger B, Morland B, Ndiaye A, Doz F, Kalifa G, Geoffray A, et al, Innovative Therapies for Children with Cancer European Consortium (2009) Target-driven exploratory study of imatinib mesylate in children with solid malignancies by the Innovative Therapies for Children with Cancer (ITCC) European Consortium. Eur J Cancer 45:2342–2351
Hasselblatt M, Thomas C, Hovestadt V et al (2016) Poorly differentiated chordoma with SMARCB1/INI1 loss: a distinct molecular entity with dismal prognosis. Acta Neuropathol 132(1):149–151
Hoch BL, Nielsen GP, Liebsch NJRA (2006) Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 30(7):811–818
Hof H, Welzel T, Debus J (2006) Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma. Onkologie 29:572–574
Hug EB (2001) Review of skull base chordomas: prognostic factors and long-term results of proton-beam radiotherapy. Neurosurg Focus 10:E11
Lui YW, Dasari SB, Young RJ (2010) Sphenoid masses in children: radiologic differential diagnosis with pathologic correlation. AJNR Am J Neuroradiol 32(4):617–626
McMaster ML, Goldstein AM, Bromley CM et al (2001) Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 12:1–11
McMaster ML, Goldstein AM, Parry DM (2011) Clinical features distinguish childhood chordoma associated with tuberous sclerosis complex (TSC) from chordoma in the general paediatric population. J Med Genet 48(7):444–449
Meng T, Jin J, Jiang C et al (2019) Molecular targeted therapy in the treatment of chordoma: a systematic review. Front Oncol 9:30
Sekhar LN, Pranatartiharan R, Chanda A et al (2001) Chordomas and chondrosarcomas of the skull base: results and complications of surgical management. Neurosurg Focus 10(3):E2
Stacchiotti S, Marrari A, Tamborini E et al (2009) Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol 20(11):1886–1894
Stacchiotti S, Longhi A, Ferraresi V et al (2012) Phase II study of imatinib in advanced chordoma. J Clin Oncol 30(9):914–920
Stacchiotti S, Sommer J, Chordoma Global Consensus Group (2015) Building a global consensus approach to chordoma: a position paper from the medical and patient community. Lancet Oncol 16(2):e71–e83
Tzortzidis F, Elahi F, Wright D, Natarajan SK, Sekhar LN (2006) Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 59:230–237
Vujovic S, Henderson S, Presneau N et al (2006) Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas. J Pathol 209(2):157–165
Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ (2012) Chordoma: current concepts, management, and future directions. Lancet Oncol 13(2):e69–e76
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Bisogno, G. (2022). Chordoma. In: Schneider, D.T., Brecht, I.B., Olson, T.A., Ferrari, A. (eds) Rare Tumors in Children and Adolescents. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-030-92071-5_38
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DOI: https://doi.org/10.1007/978-3-030-92071-5_38
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