Journal of Neuro-Oncology

, Volume 103, Issue 3, pp 657–662 | Cite as

The role of chemotherapy in pediatric clival chordomas

  • Girish DhallEmail author
  • Mercedes Traverso
  • Jonathan L. Finlay
  • Lisa Shane
  • Ignacio Gonzalez-Gomez
  • Rima Jubran
Clinical Study – Patient Study


The purpose of this retrospective study was to review the role of chemotherapy in the treatment, management and outcome of children diagnosed with clival chordomas. The medical records of six pediatric chordoma patients diagnosed at Childrens Hospital Los Angeles between 1995 and 2005 were reviewed. Of the six patients reviewed, all underwent an initial surgical resection. Following resection, three received a combination of chemotherapy and radiation therapy, two received chemotherapy alone and one patient refused both forms of therapy; this patient expired of progressive tumor. One patient developed acute monoblastic leukemia (M5a subtype) and died of intracranial hemorrhage during induction chemotherapy, 39 months after initial diagnosis. MRI of brain and spine showed disease progression shortly before his death. Two patients who received chemotherapy only after surgery, one patient who received chemotherapy at relapse following irradiation and one patient who received irradiation followed by chemotherapy are alive with stable radiographic abnormalities at a median follow-up of 9 years from diagnosis (range: 6–13 years). Chemotherapeutic agents included ifosfamide and etoposide in all four surviving patients. Chemotherapy with ifosfamide and etoposide may have a role in the treatment of pediatric clival chordomas when used alone or in combination with irradiation.


Chemotherapy Pediatric Chordomas 


  1. 1.
    Ho KL (1985) Ecchordosis physaliphora and chordoma: a comparative ultrastructural study. Clin Neuropathol 4:77–86PubMedGoogle Scholar
  2. 2.
    McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Perry DM (2001) Chordoma: incidence and survival patterns in the United States, 1973–1995. Cancer Causes Control 12:1–11PubMedCrossRefGoogle Scholar
  3. 3.
    Newton HB (2006) Chordomas. In: Raghavan D, Brecher ML, Johnson DH et al (eds) Textbook of uncommon cancer, 3rd edn. Wiley, Canada, pp 614–625CrossRefGoogle Scholar
  4. 4.
    Mirra J, Nelson S, Della Rocca C et al (2002) Chordoma. In: Fletcher CD, Unni K, Mertens F (eds) Pathology and genetics of tumors of soft tissue and bone. IARC Press, Lyon, pp 316–317Google Scholar
  5. 5.
    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
  6. 6.
    Chambers PW, Schwinn CP (1979) Chordoma. A clinicopathologic study of metastasis. Am J Clin Pathol 72:765–776PubMedGoogle Scholar
  7. 7.
    Higinbotham NL, Phillips RF, Farr HW, Hustu HO (1967) Chordoma. Thirty-five-year study at memorial hospital. Cancer 20:1841–1850PubMedCrossRefGoogle Scholar
  8. 8.
    Lanzino G, Dumont AS, Lopes MB, Laws ER Jr (2001) Skull base chordomas: overview of disease, management options, and outcome. Neurosurg Focus 10:E12PubMedCrossRefGoogle Scholar
  9. 9.
    Casali PG, Stacchiotti S, Sangalli C, Olmi P, Gronchi A (2007) Chordoma. Curr Opin Oncol 19:367–370PubMedCrossRefGoogle Scholar
  10. 10.
    Forsyth PA, Cascino TL, Shaw EG, Scheithauer BW, O’Fallon JR, Dozier JC, Piepgras DG (1993) Intracranial chordomas: a clinicopathological and prognostic study of 51 cases. J Neurosurg 78:741–747PubMedCrossRefGoogle Scholar
  11. 11.
    Fuchs B, Dickey ID, Yaszemski MJ, Inwards CY, Sim FH (2005) Operative management of sacral chordoma. J Bone Joint Surg Am 87:2211–2216PubMedCrossRefGoogle Scholar
  12. 12.
    Tai PT, Craighead P, Bagdon F (1995) Optimization of radiotherapy for patients with cranial chordoma. A review of dose-response ratios for photon techniques. Cancer 75:749–756PubMedCrossRefGoogle Scholar
  13. 13.
    Cummings BJ, Hodson DI, Bush RS (1983) Chordoma: the results of megavoltage radiation therapy. Int J Radiat Oncol Biol Phys 9:633–642PubMedCrossRefGoogle Scholar
  14. 14.
    Hug EB, Sweeney RA, Nurre PM, Holloway KC, Slater JD, Munzenrider JE (2002) Proton radiotherapy in management of pediatric base of skull tumors. Int J Radiat Oncol Biol Phys 52:1017–1024PubMedCrossRefGoogle Scholar
  15. 15.
    Fleming GF, Heimann PS, Stephens JK, Simon MA, Ferguson MK, Benjamin DS, Samuels BL (1993) Dedifferentiated chordoma. Response to aggressive chemotherapy in two cases. Cancer 72:714–718PubMedCrossRefGoogle Scholar
  16. 16.
    Scimeca PG, James-Herry AG, Black KS, Kahn E, Weinblatt ME (1996) Chemotherapeutic treatment of malignant chordoma in children. J Pediatr Hematol Oncol 18:237–240PubMedCrossRefGoogle Scholar
  17. 17.
    Borba LA, Al-Mefty O, Mrak RE, Suen J (1996) Cranial chordomas in children and adolescents. J Neurosurg 84:584–591PubMedCrossRefGoogle Scholar
  18. 18.
    Hoch BL, Nielsen GP, Liebsch NJ, Rosenberg AE (2006) Base of skull chordomas in children and adolescents: a clinicopathologic study of 73 cases. Am J Surg Pathol 30:811–818PubMedCrossRefGoogle Scholar
  19. 19.
    Ridenour RV, Ahrens WA, Folpe AL, Miller DV (2010) Clinical and histopathologic features of chordomas in children and young adults. Pediatr Dev Pathol 13(1):9–17PubMedCrossRefGoogle Scholar
  20. 20.
    Arnold H, Herrmann HD (1986) Skull base chordoma with cavernous sinus involvement. Partial or radical tumour-removal? Acta Neurochir (Wien) 83:31–37CrossRefGoogle Scholar
  21. 21.
    Tzortzidis F, Elahi F, Wright D, Natarajan SK, Shekhar LN (2006) Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 59:230–237 discussion 230–237PubMedCrossRefGoogle Scholar
  22. 22.
    Taylor RE (2003) Proton radiotherapy for paediatric tumours: potential areas for clinical research. Clin Oncol (R Coll Radiol) 15:S32–S36Google Scholar
  23. 23.
    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 37:185–191PubMedCrossRefGoogle Scholar
  24. 24.
    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
  25. 25.
    Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH (2007) Chordoma: the nonsarcoma primary bone tumor. Oncologist 12:1344–1350PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC. 2010

Authors and Affiliations

  • Girish Dhall
    • 1
    • 2
    Email author
  • Mercedes Traverso
    • 1
  • Jonathan L. Finlay
    • 1
  • Lisa Shane
    • 1
  • Ignacio Gonzalez-Gomez
    • 1
  • Rima Jubran
    • 1
  1. 1.Neural Tumors Program, The Division of Pediatric Hematology-Oncology and Department of Pathology at Childrens Hospital Los AngelesLos AngelesUSA
  2. 2.Neural Tumors Program, Childrens Hospital Los Angeles, Keck School of MedicineUniversity of Southern CaliforniaLos AngelesUSA

Personalised recommendations