Child's Nervous System

, Volume 26, Issue 6, pp 835–840 | Cite as

Pediatric cervical chordoma: report of two cases and a review of the current literature

  • Gwi Hyun Choi
  • Moon-Sool Yang
  • Do Heum Yoon
  • Hyun Chyul Shin
  • Keung Nyun Kim
  • Seong Yi
  • Dong Yeop Lee
  • Poong Gi Ahn
  • Yoon HaEmail author
Case Report



The majority of chordomas occur between the fifth and seventh decades of life and are thus extremely rare in children, which account for less than 5% of all spinal chordoma cases.

Case report

We report on the development of this rare condition in two boys aged 7 and 10 years. One patient presented with a palpable neck mass and dysphagia, while the other presented with posterior neck pain. Radiological studies revealed an extensive mass in the cervical vertebrae and paravertebral soft tissue of both patients. The tumors were subtotally removed in an attempt to improve the success of adjuvant proton beam radiotherapy. Pathological examination, which included immunohistochemical staining, revealed chordoma of the cervical spine in both patients.


Although en bloc resection is the ideal modality for treatment of chordoma, such a procedure is often associated with a significant risk of surgical morbidity due to the tumor location. Therefore, piecemeal resection followed by postoperative adjuvant radiotherapy, including proton radiotherapy or radiosurgery, should be considered in such cases.


Chordoma Cervical spine Childhood 



This study was supported by a Korea Research Foundation grant (KRF-2008-313-H00022) funded by the Korean government and a grant from the Stem Cell Research Center (SC-4180) of the 21st Century Frontier Research Program funded by the Ministry of Education, Science and Technology, Republic of Korea.


The authors report no conflicts of interest concerning the materials or methods used in this study or the findings specified in this paper.


  1. 1.
    Bas T, Bas P, Prieto M, Ramos V, Bas JL, Espinosa C (1994) A lumbar chordoma treated with a wide resection. Eur Spine J 3:115–117CrossRefPubMedGoogle Scholar
  2. 2.
    Carpentier A, Polivka M, Blanquet A, Lot G, George B (2002) Suboccipital and cervical chordomas: the value of aggressive treatment at first presentation of the disease. J Neurosurg 97:1070–1077CrossRefPubMedGoogle Scholar
  3. 3.
    Mendenhall WM, Mendenhall CM, Lewis SB, Villaret DB, Mendenhall NP (2005) Skull base chordoma. Head Neck 27:159–165CrossRefPubMedGoogle Scholar
  4. 4.
    Murali R, Rovit RL, Benjamin MV (1981) Chordoma of the cervical spine. Neurosurgery 9:253–256CrossRefPubMedGoogle Scholar
  5. 5.
    McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM (2001) Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 12:1–11CrossRefPubMedGoogle Scholar
  6. 6.
    O’Neill P, Bell BA, Miller JD, Jacobson I, Guthrie W (1985) Fifty years of experience with chordomas in southeast Scotland. Neurosurgery 16:166–170CrossRefPubMedGoogle Scholar
  7. 7.
    Sundaresan N, Galicich JH, Chu FC, Huvos AG (1979) Spinal chordomas. J Neurosurg 50:312–319CrossRefPubMedGoogle Scholar
  8. 8.
    Noël G, Feuvret L, Calugaru V, Dhermain F, Mammar H, Haie-Méder C, Ponvert D, Hasboun D, Ferrand R, Nauraye C, Boisserie G, Beaudré A, Gaboriaud G, Mazal A, Habrand JL, Mazeron JJ (2005) 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 (Madr) 44:700–708CrossRefGoogle Scholar
  9. 9.
    Azzarelli A, Quagliuolo V, Cerasoli S, Zucali R, Bignami P, Mazzaferro V, Dossena G, Gennari L (1988) Chordoma: natural history and treatment results in 33 cases. J Surg Oncol 37:185–191CrossRefPubMedGoogle Scholar
  10. 10.
    Cummings BJ, Hodson DI, Bush RS (1983) Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys 9:633–642PubMedGoogle Scholar
  11. 11.
    Handa J, Suzuki F, Nioka H, Koyama T (1987) Clivus chordoma in childhood. Surg Neurol 28:58–62CrossRefPubMedGoogle Scholar
  12. 12.
    Burger PC, Makek M, Kleihues P (1986) Tissue polypeptide antigen staining of the chordoma and notochordal remnants. Acta Neuropathol 70:269–272CrossRefPubMedGoogle Scholar
  13. 13.
    Salisbury JR (1993) The pathology of the human notochord. J Pathol 171:253–255CrossRefPubMedGoogle Scholar
  14. 14.
    Chevalier X, Voisin MC, Brugières P, Ducoup-Lepointe H, Avouac B, Marty M, Martigny J, Hernigou PH, Goutallier D, Villiaumey J (1990) Chordoma of the mobile spine. Report of 9 cases. Review of the literature. Rev Rhum Mal Osteoartic 57:767–778PubMedGoogle Scholar
  15. 15.
    Mortelé B, Lemmerling M, Mortelé K, Verstraete K, Defreyne L, Kunnen M, Vandekerckhove T (2000) Cervical chordoma with vertebral artery encasement mimicking neurofibroma: MRI findings. Eur Radiol 10:967–969CrossRefPubMedGoogle Scholar
  16. 16.
    Wippold FJ, Koeller KK, Smirniotopoulos JG (1999) Clinical and imaging features of cervical chordoma. AJR Am J Roentgenol 172:1423–1426PubMedGoogle Scholar
  17. 17.
    Manzone P, Fiore N, Forlino D, Alcala M, Cabrera CF (1998) Chordoma of the lumbar L2 vertebra: case report and review of the literature. Eur Spine J 7:252–256CrossRefPubMedGoogle Scholar
  18. 18.
    Bjornsson J, Wold LE, Ebersold MJ, Laws ER (1993) Chordoma of the mobile spine. A clinicopathologic analysis of 40 patients. Cancer 71:735–740CrossRefPubMedGoogle Scholar
  19. 19.
    Meyers SP, Hirsch WL, Curtin HD, Barnes L, Sekhar LN, Sen C (1992) Chordomas of the skull base: MR features. AJNR Am J Neuroradiol 13:1627–1636PubMedGoogle Scholar
  20. 20.
    Sundaresan N (1986) Chordomas. Clin Orthop Relat Res 204:135–142PubMedGoogle Scholar
  21. 21.
    Godard J, Jacquet G, Pageaut G, Farhat O, Steimle R (1990) Cervical chordoma. Apropos of a case. Neurochirurgie 36:388–393PubMedGoogle Scholar
  22. 22.
    Boriani S, Chevalley F, Weinstein JN, Biagini R, Campanacci L, De Iure F, Piccill P (1996) Chordoma of the spine above the sacrum. Treatment and outcome in 21 cases. Spine 21:1569–1577CrossRefPubMedGoogle Scholar
  23. 23.
    Healey JH, Lane JM (1989) Chordoma: a critical review of diagnosis and treatment. Orthop Clin North Am 20:417–426PubMedGoogle Scholar
  24. 24.
    Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW (1973) Chordomas and cartilaginous tumors at the skull base. Cancer 32:410–420CrossRefPubMedGoogle Scholar
  25. 25.
    Firooznia H, Golimbu C, Rafii M, Reede DL, Kricheff II, Bjorkengren A (1986) Computed tomography of spinal chordomas. J Comput Tomogr 10:45–50CrossRefPubMedGoogle Scholar
  26. 26.
    Yuh WT, Lozano RL, Flickinger FW, Sato Y, Kao SS, Menezes AH (1989) Lumbar epidural chordoma: MR findings. J Comput Assist Tomogr 13:508–510CrossRefPubMedGoogle Scholar
  27. 27.
    Murphy JM, Wallis F, Toland J, Toner M, Wilson GF (1998) CT and MRI appearances of a thoracic chordoma. Eur Radiol 8:1677–1679CrossRefPubMedGoogle Scholar
  28. 28.
    Smolders D, Wang X, Drevelengas A, Vanhoenacker F, De Schepper AM (2003) Value of MRI in the diagnosis of non-clival, non-sacral chordoma. Skeletal Radiol 32:343–350PubMedGoogle Scholar
  29. 29.
    Murphey MD, Andrews CL, Flemming DJ, Temple HT, Smith WS, Smirniotopoulos JG (1996) From the archives of the AFIP. Primary tumors of the spine: radiologic pathologic correlation. Radiographics 16:1131–1158PubMedGoogle Scholar
  30. 30.
    D’Haen B, De Jaegere T, Goffin J, Dom R, Demaerel P, Plets C (1995) Chordoma of the lower cervical spine. Clin Neurol Neurosurg 97:245–248CrossRefPubMedGoogle Scholar
  31. 31.
    Eriksson B, Gunterberg B, Kindblom LG (1981) Chordoma. A clinicopathologic and prognostic study of a Swedish national series. Acta Orthop Scand 52:49–58PubMedCrossRefGoogle Scholar
  32. 32.
    Mindell ER (1981) Chordoma. J Bone Joint Surg Am 63:501–505PubMedGoogle Scholar
  33. 33.
    Walaas L, Kindblom LG (1991) Fine-needle aspiration biopsy in the preoperative diagnosis of chordoma: a study of 17 cases with application of electron microscopic, histochemical, and immunocytochemical examination. Hum Pathol 22:22–28CrossRefPubMedGoogle Scholar
  34. 34.
    Baratti D, Gronchi A, Pennacchioli E, Lozza L, Colecchia M, Fiore M, Santinami M (2003) Chordoma: natural history and results in 28 patients treated at a single institution. Ann Surg Oncol 10:291–296CrossRefPubMedGoogle Scholar
  35. 35.
    Chambers PW, Schwinn CP (1979) Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 72:765–776PubMedGoogle Scholar
  36. 36.
    Delank KS, Kriegsmann J, Drees P, Eckardt A, Eysel P (2002) Metastasizing chordoma of the lumbar spine. Eur Spine J 11:167–171CrossRefPubMedGoogle Scholar
  37. 37.
    Higinbotham NL, Phillips RF, Farr HW, Hustu HO (1967) Chordoma. Thirty-five-year study at Memorial Hospital. Cancer 20:1841–1850CrossRefPubMedGoogle Scholar
  38. 38.
    Coffin CM, Swanson PE, Wick MR, Dehner LP (1993) Chordoma in childhood and adolescence. A clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 117:927–933PubMedGoogle Scholar
  39. 39.
    Sibley RK, Day DL, Dehner LP, Trueworthy RC (1987) Metastasizing chordoma in early childhood: a pathological and immunohistochemical study with review of the literature. Pediatr Pathol 7:287–301CrossRefPubMedGoogle Scholar
  40. 40.
    Boriani S, Biagini R, De Iure F, Di Fiore M, Gamberini G, Zanoni A (1994) Lumbar vertebrectomy for the treatment of bone tumors: surgical technique. Chir Organi Mov 79:163–173PubMedGoogle Scholar
  41. 41.
    Stener B (1989) Complete removal of vertebrae for extirpation of tumors. A 20-year experience. Clin Orthop Relat Res 245:72–82PubMedGoogle Scholar
  42. 42.
    Booi GA, van Horn JR (1987) Cervical chordoma. A case report. Acta Orthop Belg 53:520–522PubMedGoogle Scholar
  43. 43.
    Catton C, O’Sullivan B, Bell R, Laperriere N, Cummings B, Fornasier V, Wunder J (1996) Chordoma: long-term follow-up after radical photon irradiation. Radiother Oncol 41:67–72PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Gwi Hyun Choi
    • 1
  • Moon-Sool Yang
    • 1
  • Do Heum Yoon
    • 1
  • Hyun Chyul Shin
    • 2
  • Keung Nyun Kim
    • 1
  • Seong Yi
    • 1
  • Dong Yeop Lee
    • 1
  • Poong Gi Ahn
    • 1
  • Yoon Ha
    • 1
    Email author
  1. 1.Department of Neurosurgery, Spine and Spinal Cord Institute, College of MedicineYonsei UniversitySeoulRepublic of Korea
  2. 2.Department of Neurosurgery, Kangpuk Samsung Hospital, College of MedicineSungkyunkwan UniversitySeoulSouth Korea

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