Intramedullary Spinal Cord Tumors

  • A. Quinones-Hinojosa
  • M. Gulati
  • M. H. Schmidt
Part of the Pediatric Oncology book series (PEDIATRICO)


Intramedullary spinal cord tumors (IMSCTs) are rare and account for only 4 to 6% of all pediatric central nervous system tumors (DeSousa et al. 1979). Approximately 100 to 200 cases are diagnosed each year in the US (Constantini and Epstein 1995), representing an incidence of less than 1 in 100,000. Pediatric IMSCTs are usually located in the cervical or thoracic spine (90 to 95%) and occur infrequently in the lumbar spine (Epstein and Ragheb 1994; Goh et al. 2000). There is an equal male and female distribution in the reported pediatric series (Brotchi et al. 1992; Goh et al. 1997; Zileli et al. 1996).


Spinal Cord Pilocytic Astrocytoma Postoperative Magnetic Resonance Imaging Spinal Cord Tumor Intramedullary Tumor 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Abbott R (1993) Tumors of the medulla. Neurosurg Clin North Am 4:519–527Google Scholar
  2. Allen JC, Aviner S, Yates Al, Boyett JM, Cherlow JM, Turski PA, Epstein F, Finlay JL (1998) Treatment of high-grade spinal cord astrocytoma of childhood with “8-in-1” chemotherapy and radiotherapy: a pilot study of CCG-945. Children’s Cancer Group. J Neurosurg 88: 215–220PubMedCrossRefGoogle Scholar
  3. Avizonis VN, Fuller DB, Thomson JW, Walker MJ, Nilsson DE, Menlove RL (1992) Late effects following central nervous system radiation in a pediatric population. Neuropediatrics 23: 228–234PubMedCrossRefGoogle Scholar
  4. Baleriaux DL (1999) Spinal cord tumors. Eur Radiol 9: 12521258Google Scholar
  5. Birch BD, Johnson JP, Parsa A, Desai RD, Yoon JT, Lycette CA, Li YM, Bruce JN (1996) Frequent type 2 neurofibromatosis gene transcript mutations in sporadic intramedullary spinal cord ependymomas. Neurosurgery 39:135–140Google Scholar
  6. Brotchi J, Dewitte O, Levivier M, Baleriaux D, Vandesteene A, Raftopoulos C, Flament-Durand J, Noterman J (1991) A survey of 65 tumors within the spinal cord: surgical results and the importance of preoperative magnetic resonance imaging. Neurosurgery 29: 651–656PubMedCrossRefGoogle Scholar
  7. Brotchi J, Noterman J, Baleriaux D (1992) Surgery of intramedullary spinal cord tumours. Acta Neurochir (Wien) 116ä76178Google Scholar
  8. Brunberg JA, DiPietro MA, Venes JL, Dauser RC, Muraszko KM, Berkey GS, D’Amato CJ, Rubin JM (1991) Intramedullary lesions of the pediatric spinal cord: correlation of findings from MR imaging, intraoperative sonography, surgery, and histologic study. Radiology 181:573–579Google Scholar
  9. Cohen AR, Wisoff JH, Allen JC, Epstein F (1989) Malignant astrocytomas of the spinal cord. J Neurosurg 7o:5o-54Google Scholar
  10. Constantini S, Epstein F (1995) Intraspinal tumors in children and infants. In: Youmans J (ed) Neurological surgery. Saunders, Philadelphia, pp 3132–3167Google Scholar
  11. Constantini S, Houten J, Miller DC, Freed D, Ozek MM, Rorke LB, Allen IC, Epstein FJ (1996) Intramedullary spinal cord tumors in children under the age of 3 years. J Neurosurg 85: 1036–1043PubMedCrossRefGoogle Scholar
  12. Cooper PR (1989) Outcome after operative treatment of intramedullary spinal cord tumors in adults: intermediate and long-term results in 51 patients. Neurosurgery 25855859Google Scholar
  13. Cristante L, Herrmann HD (1994) Surgical management of intramedullary spinal cord tumors: functional outcome and sources of morbidity. Neurosurgery 35:69–74Google Scholar
  14. DeSousa AL, Kalsbeck JE, Mealey J Jr, Campbell RL, Hockey A (1979) Intraspinal tumors in children. A review of 81 cases. J Neurosurg 51:437–445Google Scholar
  15. Doireau V, Grill J, Zerah M, Lellouch-Tubiana A, Couanet D, Chastagner P, Marchai JC, Grignon Y, Chouffai Z, Kalifa C (1999) Chemotherapy for unresectable and recurrent intramedullary glial tumours in children. Brain Tumours Subcommittee of the French Society of Paediatric Oncology (SFOP). Br J Cancer 81: 835–84oPubMedCrossRefGoogle Scholar
  16. Ebert C, von Haken M, Meyer-Puttlitz B, Wiestler OD, Reifen-berger G, Pietsch T, von Deimling A (1999) Molecular genetic analysis of ependymal tumors. NF2 mutations and chromosome 22q loss occur preferentially in intramedullary spinal ependymomas. Am J Pathol 155: 627–632Google Scholar
  17. Egelhoff JC, Bates DJ, Ross JS, Rothner AD, Cohen BH (1992) Spinal MR findings in neurofibromatosis types 1 and 2. AJNR Am J Neuroradiol 13: 1071–1077PubMedGoogle Scholar
  18. Epstein F (1986) Intraaxial tumor of the spinal cord and cervicomedullary junction in children. In: Hoffman HJ (ed) Advances in pediatric neurosurgery. Hanley and Belfus, Philadelphia, pp 17–34Google Scholar
  19. Epstein F, Ragheb J (1994) Intramedullary tumors of the spinal cord. In: Cheek W (ed) Pediatric neurosurgery: surgery of the developing nervous system. Saunders, Philadelphia, pp 446-457Google Scholar
  20. Epstein FJ, Farmer JP, Freed D (1993) Adult intramedullary spinal cord ependymomas: the result of surgery in 38 patients. J Neurosurg 79: 204–209PubMedCrossRefGoogle Scholar
  21. Fine MJ, Kricheff II, Freed D, Epstein FJ (1995) Spinal cord ependymomas: MR imaging features. Radiology 197:655658Google Scholar
  22. Frappaz D, Ricci AC, Kohler R, Bret P, Mottolese C (1999) Diffuse brain stem tumor in an adolescent with multiple enchondromatosis (011ier’s disease). Childs Nery Syst 15: 22 2225Google Scholar
  23. Goh K, Muszynski C, Teo J, Constantini S, Epstein F (2000) Excision of spinal intramedullary tumors. In: Kaye A, Black P (eds) Operative neurosurgery, Churchill Livingstone: London, pp 1947–1959Google Scholar
  24. Goh KY, Velasquez L, Epstein FJ (1997) Pediatric intramedullary spinal cord tumors: is surgery alone enough? Pediatr Neurosurg 27:34–39Google Scholar
  25. Grabb PA, Kelly DR, Fulmer BB, Palmer C (1996) Radiation-induced glioma of the spinal cord. Pediatr Neurosurg 25: 214219Google Scholar
  26. Guidetti B, Mercuri S, Vagnozzi R (1981) Long-term results of the surgical treatment of 129 intramedullary spinal gliomas. J Neurosurg 54:323–330Google Scholar
  27. Hassall TE, Mitchell AE, Ashley DM (2001) Carboplatin chemotherapy for progressive intramedullary spinal cord low-grade gliomas in children: three case studies and a review of the literature. Neurooncology 3:251–257Google Scholar
  28. Hoshimaru M, Koyama T, Hashimoto N, Kikuchi H (1999) Results of microsurgical treatment for intramedullary spinal cord ependymomas: analysis of 36 cases. Neurosurgery 44: 264–269Google Scholar
  29. Houten JK, Weiner HL (2000) Pediatric intramedullary spinal cord tumors: special considerations. J Neurooncol 47: 225230Google Scholar
  30. Huang H, Reis R, Yonekawa Y, Lopes JM, Kleihues P, Ohgaki H (1999) Identification in human brain tumors of DNA sequences specific for SV40 large T antigen. Brain Pathol 9:33-42 Google Scholar
  31. Isaacson SR (2000) Radiation therapy and the management of intramedullary spinal cord tumors. J Neurooncol 47: 231238Google Scholar
  32. Kleihues P, Cavenee WK (eds) (2000) Pathology and genetics of tumours of the nervous system. IARC Press, LyonGoogle Scholar
  33. Kothbauer K, Deletis V, Epstein FJ (1997) Intraoperative spinal cord monitoring for intramedullary surgery: an essential adjunct. Pediatr Neurosurg 26:247–254Google Scholar
  34. Kramer DL, Parmiter AH, Rorke LB, Sutton LN, Biegel JA (1998) Molecular cytogenetic studies of pediatric ependymomas. J Neurooncol 37:25–33Google Scholar
  35. Lashford LS, Campbell RH, Gattamaneni HR, Robinson K, Walker D, Bailey C (1996) An intensive multiagent chemotherapy regimen for brain tumours occurring in very young children. Arch Dis Child 74: 219–223PubMedCrossRefGoogle Scholar
  36. Lee M, Rezai AR, Freed D, Epstein FJ (1996) Intramedullary spinal cord tumors in neurofibromatosis. Neurosurgery 38: 3237Google Scholar
  37. Linstadt DE, Wara WM, Leibel SA, Gutin PH, Wilson CB, Sheline GE (1989) Postoperative radiotherapy of primary spinal cord tumors. Int J Radiat Oncol Biol Phys 16:1397–1403Google Scholar
  38. Lowis SP, Pizer BL, Coakham H, Nelson RJ, Bouffet E (1998) Chemotherapy for spinal cord astrocytoma: can natural history be modified? Childs Nery Syst 14: 317–321CrossRefGoogle Scholar
  39. Maiuri F, Iaconetta G, Gallicchio B, Stella L (2000) Intraoperative sonography for spinal tumors. Correlations with MR findings and surgery. J Neurosurg Sci 44: 115–122Google Scholar
  40. McCormick PC, Torres R, Post KD, Stein BM (1990) Intramedullary ependymoma of the spinal cord. J Neurosurg 72:523532Google Scholar
  41. McLaughlin MP, Buatti JM, Marcus RB Jr, Maria BL, Mickle PJ, Kedar A (1998) Outcome after radiotherapy of primary spinal cord glial tumors. Radiat Oncol Invest 6: 276–280CrossRefGoogle Scholar
  42. McLaughlin MP, Marcus RB Jr, Buatti JM, McCollough WM, Mickle JP, Kedar A, Maria BL, Million RR (1998) Ependymoma: results, prognostic factors and treatment recommendations. Int J Radiat Oncol Biol Phys 40:845–850Google Scholar
  43. Mellon CD, Carter JE, Owen DB (1988) 011ier’s disease and Maffucci’s syndrome: distinct entities or a continuum. Case report: enchondromatosis complicated by an intracranial glioma. J Neurol 235:376-378Google Scholar
  44. Merchant TE, Kiehna EN, Thompson SJ, Heideman R, Sanford RA, Kun LE (2000) Pediatric low-grade and ependymal spinal cord tumors. Pediatr Neurosurg 32: 30–36PubMedCrossRefGoogle Scholar
  45. Miller DC (2000) Surgical pathology of intramedullary spinal cord neoplasms. J Neurooncol 47: 189–194PubMedCrossRefGoogle Scholar
  46. Miyazawa N, Hida K, Iwasaki Y, Koyanagi I, Abe H (2000) MRI at 1.5 T of intramedullary ependymoma and classification of pattern of contrast enhancement. Neuroradiology 42: 828–832PubMedCrossRefGoogle Scholar
  47. Nagib MG, O’Fallon MT (1997) Myxopapillary ependymoma of the conus medullaris and filum terminale in the pediatric age group. Pediatr Neurosurg 26: 2–7PubMedCrossRefGoogle Scholar
  48. Osborn AG (1994) Diagnostic neuroradiology. Mosby, St LouisGoogle Scholar
  49. Prados MD, Edwards MS, Rabbitt J, Lamborn K, Davis RL, Levin VA (1997) Treatment of pediatric low-grade gliomas with a nitrosourea-based multiagent chemotherapy regimen. J Neurooncol 32: 235–241PubMedCrossRefGoogle Scholar
  50. Przybylski GJ, Albright AL, Martinez AJ (1997) Spinal cord astrocytomas: long-term results comparing treatments in children. Childs Nery Syst 13:375–382Google Scholar
  51. Quinones-Hinojosa A, Lyon R, Gulati M, Lyon R, Gupta N,Yingling C (2002) Spinal cord mapping as an adjunct for resection of intramedullary tumors: Surgical technique with case illustrations. Neurosurgery 51: 1199–1206Google Scholar
  52. Raghavendra BN, Epstein FJ, McCleary L (1984) Intramedullary spinal cord tumors in children: localization by intraoperative sonography. AJNR Am J Neuroradiol 5:395–397Google Scholar
  53. Raimondi AJ, Gutierrez FA, di Rocco C (1976) Laminotomy and total reconstruction of the posterior spinal arch for spinal canal surgery in childhood. J Neurosurg 45:555–560Google Scholar
  54. Ransom DT, Ritland SR, Kimmel DW, Moertel CA, Dahl RJ, Scheithauer BW, Kelly PJ, Jenkins RB (1992) Cytogenetic and loss of heterozygosity studies in ependymomas, pilocytic astrocytomas, and oligodendrogliomas. Genes Chromosomes Cancer 5:348–356Google Scholar
  55. Ransom DT, Ritland SR, Moertel CA, Dahl RJ, O’Fallon JR, Scheithauer BW, Kimmel DW, Kelly PJ, Olopade 0I, Diaz MO et al (1992) Correlation of cytogenetic analysis and loss of heterozygosity studies in human diffuse astrocytomas and mixed oligo-astrocytomas. Genes Chromosomes Cancer 5:357–374Google Scholar
  56. Reimer R, Onofrio BM (1985) Astrocytomas of the spinal cord in children and adolescents. J Neurosurg 63: 669–675PubMedCrossRefGoogle Scholar
  57. Robertson PL, Allen JC, Abbott IR, Miller DC, Fidel J, Epstein FJ (1994) Cervicomedullary tumors in children: a distinct subset of brainstem gliomas. Neurology 44x798–1803Google Scholar
  58. Rossitch E Jr, Zeidman SM, Burger PC, Curnes JT, Harsh C, An-scher M, Oakes WI (1990) Clinical and pathological analysis of spinal cord astrocytomas in children. Neurosurgery 27x93–196Google Scholar
  59. Salewski H, Bayer TA, Eidhoff U, Preuss U, Weggen S, Scheidt-mann KH (1999) Increased oncogenicity of subclones of SV4o large T-induced neuroectodermal tumor cell lines after loss of large T expression and concomitant mutation in p53. Cancer Res 59: 1980–1986Google Scholar
  60. Sandler HM, Papadopoulos SM, Thornton AF Jr, Ross DA (1992) Spinal cord astrocytomas: results of therapy. Neurosurgery 30: 490–493PubMedCrossRefGoogle Scholar
  61. Steinbok P, Cochrane DD, Poskitt K (1992) Intramedullary spinal cord tumors in children. Neurosurg Clin North Am 3:931–945Google Scholar
  62. Van Nielen KM, de Jong BM (1999) A case of 011ier’s disease associated with two intracerebral low-grade gliomas. Clin Neurol Neurosurg 101:106–110Google Scholar
  63. Von Deimling A, Louis DN, Menon AG, von Ammon K, Petersen I, Ellison D, Wiestler OD, Seizinger BR (1993) Deletions on the long arm of chromosome 17 in pilocytic astrocytoma. Acta Neuropathol (Berl) 86: 81–85Google Scholar
  64. Von Haken MS, White EC, Daneshvar-Shyesther L, Sib S, Choi E, Kalra R, Cogen PH (1996) Molecular genetic analysis of chromosome arm 17 and chromosome arm 22q DNA sequences in sporadic pediatric ependymomas. Genes Chromosomes Cancer 17:37–44Google Scholar
  65. Wen BC, Hussey DH, Hitchon PW, Schelper RL, Vigliotti AP, Doornbos JF,VanGilder JC (1991) The role of radiation therapy in the management of ependymomas of the spinal cord. Int J Radiat Oncol Biot Phys 20: 781–786Google Scholar
  66. Zileli M, Coskun E, Ozdamar N, Ovul I, Tuncbay E, Oner K, Oktar N (1996) Surgery of intramedullary spinal cord tumors. Eur Spine] 5: 243–250CrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2004

Authors and Affiliations

  • A. Quinones-Hinojosa
  • M. Gulati
  • M. H. Schmidt

There are no affiliations available

Personalised recommendations