Abstract
Intramedullary spinal cord tumors (IMSCT) are rare lesions in the pediatric population, accounting for 4–8 % of central nervous system tumors in children (Bowers and Weprin 2003; DeSousa et al. 1979; Barker et al. 1976). They represent 55 % of intradural tumors in this age group (Yamamoto and Raffel 1999). They occur with an approximate incidence of 1 in 100,000, with 100–200 cases of pediatric IMSCTs diagnosed each year in the United States (Constantini and Epstein 1995; Constantini 1996). The majority of these lesions (nearly 60 %) are encountered in the cervical and thoracic regions and rarely involve the lumbar cord (Goh et al. 2000; Cooper 1989). They are distributed evenly between male and female patients (Goh et al. 1997). Primary glial tumors such as ependymomas and astrocytomas account for at least 80 % of IMSCTs (Cooper 1989; Cristante and Herrmann 1994; Epstein et al. 1993; Hoshimaru et al. 1999; McCormick et al. 1990b; Sandler et al. 1992). Other less common but reported pediatric IMSCT tumor types include ganglioglioma (Lang et al. 1993; Hayashi et al. 2011), oligodendroglioma (Miller 2000), and hemangioblastoma (Lang et al. 1993; Roonprapunt et al. 2001; Weil et al. 2003). Rarer still have been case reports of primitive neuroectodermal tumors (Alexiou et al. 2013), subependymoma (Miller and McCutcheon 2000), pleomorphic xanthoastrocytoma (Das et al. 2014), neurocytoma (Singh et al. 2007), schwannoma (Kim et al. 2009), teratoma (Isik et al. 2008), germinoma (Madhukar et al. 2013), and non-Hodgkin’s lymphoma (Bhushanam et al. 2014).
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Abbott R (1993) Tumors of the medulla. Neurosurg Clin N Am 4(3):519–527
Ahmed R, Menezes AH, Awe OO, Mahaney KB, Torner JC, Weinstein SL (2014a) Long-term incidence and risk factors for development of spinal deformity following resection of pediatric intramedullary spinal cord tumors. J Neurosurg Pediatr 13(6):613–621. doi:10.3171/2014.1.PEDS13317
Ahmed R, Menezes AH, Awe OO, Torner JC (2014b) Long-term disease and neurological outcomes in patients with pediatric intramedullary spinal cord tumors. J Neurosurg Pediatr 13(6):600–612. doi:10.3171/2014.1.PEDS13316
Alexiou GA, Siozos G, Stefanaki K, Moschovi M, Prodromou N (2013) Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus. J Child Neurol 28(2):246–250. doi:10.1177/0883073812441061
Allen JC, Aviner S, Yates AJ, 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(2):215–220. doi:10.3171/jns.1998.88.2.0215
Ammerlaan AC, de Bustos C, Ararou A, Buckley PG, Mantripragada KK, Verstegen MJ, Hulsebos TJ, Dumanski JP (2005) Localization of a putative low-penetrance ependymoma susceptibility locus to 22q11 using a chromosome 22 tiling-path genomic microarray. Gene Chromosome Cancer 43(4):329–338. doi:10.1002/gcc.20207
Anakwenze OA, Auerbach JD, Buck DW, Garg S, Simon SL, Sutton LN, Sponseller PD, Dormans JP (2011) The role of concurrent fusion to prevent spinal deformity after intramedullary spinal cord tumor excision in children. J Pediatr Orthop 31(5):475–479. doi:10.1097/BPO.0b013e318220bb46
Asazuma T, Toyama Y, Suzuki N, Fujimura Y, Hirabayshi K (1999) Ependymomas of the spinal cord and cauda equina: an analysis of 26 cases and a review of the literature. Spinal Cord 37(11):753–759
Auguste KI, Gupta N (2006) Pediatric intramedullary spinal cord tumors. Neurosurg Clin N Am 17(1):51–61. doi:10.1016/j.nec.2005.10.004
Baleriaux DL (1999) Spinal cord tumors. Eur Radiol 9(7):1252–1258. doi:10.1007/s003300050831
Barker DJ, Weller RO, Garfield JS (1976) Epidemiology of primary tumours of the brain and spinal cord: a regional survey in southern England. J Neurol Neurosurg Psychiatry 39(3):290–296
Beckner ME, Sasatomi E, Swalsky PA, Hamilton RL, Pollack IF, Finkelstein SD (2004) Loss of heterozygosity reveals non-VHL allelic loss in hemangioblastomas at 22q13. Hum Pathol 35(9):1105–1111
Bhushanam TV, Rajesh A, Linga VG, Uppin MS, Malik M (2014) Primary intramedullary non-Hodgkin’s lymphoma in an immunocompetent child. Spinal Cord 52(Suppl 2):S21–S23. doi:10.1038/sc.2014.96
Bijlsma EK, Voesten AM, Bijleveld EH, Troost D, Westerveld A, Merel P, Thomas G, Hulsebos TJ (1995) Molecular analysis of genetic changes in ependymomas. Gene Chromosome Cancer 13(4):272–277
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(1):135–140
Bowers DC, Weprin BE (2003) Intramedullary spinal cord tumors. Curr Treat Options Neurol 5(3):207–212
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(5):651–656, discussion 656–657
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(2):573–579. doi:10.1148/radiology.181.2.1924807
Calancie B, Harris W, Broton JG, Alexeeva N, Green BA (1998) “Threshold-level” multipulse transcranial electrical stimulation of motor cortex for intraoperative monitoring of spinal motor tracts: description of method and comparison to somatosensory evoked potential monitoring. J Neurosurg 88(3):457–470. doi:10.3171/jns.1998.88.3.0457
Chamberlain MC (2001) Recurrent intracranial ependymoma in children: salvage therapy with oral etoposide. Pediatr Neurol 24(2):117–121
Chamberlain MC (2002a) Etoposide for recurrent spinal cord ependymoma. Neurology 58(8):1310–1311
Chamberlain MC (2002b) Salvage chemotherapy for recurrent spinal cord ependymoma. Cancer 95(5):997–1002. doi:10.1002/cncr.10826
Chamberlain MC (2008) Temozolomide for recurrent low-grade spina cord gliomas in adults. Cancer 113:1019–1024
Chang UK, Choe WJ, Chung SK, Chung CK, Kim HJ (2002) Surgical outcome and prognostic factors of spinal intramedullary ependymomas in adults. J Neuro Oncol 57(2):133–139
Cheng JS, Ivan ME, Stapleton CJ, Quinones-Hinojosa A, Gupta N, Auguste KI (2014a) Intraoperative changes in transcranial motor evoked potentials and somatosensory evoked potentials predicting outcome in children with intramedullary spinal cord tumors. J Neurosurg Pediatr 13 (6):591–9. doi:10.3171/2014.2.PEDS1392
Cheng JS, Ivan ME, Stapleton CJ, Quinones-Hinojosa A, Gupta N, Auguste KI (2014b) Intraoperative changes in transcranial motor evoked potentials and somatosensory evoked potentials predicting outcome in children with intramedullary spinal cord tumors. J Neurosurg Pediatr 13(6):591–9. doi: 10.3171/2014.2.PEDS1392.
Choudhri AF, Whitehead MT, Klimo P Jr, Montgomery BK, Boop FA (2014) Diffusion tensor imaging to guide surgical planning in intramedullary spinal cord tumors in children. Neuroradiology 56(2):169–174. doi:10.1007/s00234-013-1316-9
Clover LL, Hazuka MB, Kinzie JJ (1993) Spinal cord ependymomas treated with surgery and radiation therapy. A review of 11 cases. Am J Clin Oncol 16(4):350–353
Constantini SEF (1996) Intraspinal tumors in infants and children. In: Youmans J (ed) Neurological surgery, vol 4. Saunders, Philadelphia, pp 3123–3133
Constantini S, Epstein F (1995) Intraspinal tumors in children and infants. In: Youmans J (ed) Neurological surgery. WB Saunders, Philadelphia, pp 3132–3167
Constantini S, Houten J, Miller DC, Freed D, Ozek MM, Rorke LB, Allen JC, Epstein FJ (1996) Intramedullary spinal cord tumors in children under the age of 3 years. J Neurosurg 85(6):1036–1043. doi:10.3171/jns.1996.85.6.1036
Constantini S, Miller DC, Allen JC, Rorke LB, Freed D, Epstein FJ (2000) Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg 93(2 Suppl):183–193
Cooper PR (1989) Outcome after operative treatment of intramedullary spinal cord tumors in adults: intermediate and long-term results in 51 patients. Neurosurgery 25(6):855–859
Cooper PR, Epstein F (1985) Radical resection of intramedullary spinal cord tumors in adults. Recent experience in 29 patients. J Neurosurg 63(4):492–499. doi:10.3171/jns.1985.63.4.0492
Costa P, Peretta P, Faccani G (2013) Relevance of intraoperative D wave in spine and spinal cord surgeries. Eur Spine J 22(4):840–848. doi:10.1007/s00586-012-2576-5
Cristante L, Herrmann HD (1994) Surgical management of intramedullary spinal cord tumors: functional outcome and sources of morbidity. Neurosurgery 35(1):69–74, discussion 74–66
Das S, Yip S, Hukin J, Cochrane D, Dunham C (2014) Pleomorphic xanthoastrocytoma of the spinal cord: case report and literature review. Clin Neuropathol. doi:10.5414/NP300689
DeSousa AL, Kalsbeck JE, Mealey J Jr, Campbell RL, Hockey A (1979) Intraspinal tumors in children. A review of 81 cases. J Neurosurg 51(4):437–445. doi:10.3171/jns.1979.51.4.0437
Dow G, Biggs N, Evans G, Gillespie J, Ramsden R, King A (2005) Spinal tumors in neurofibromatosis type 2. Is emerging knowledge of genotype predictive of natural history? J Neurosurg Spine 2(5):574–579. doi:10.3171/spi.2005.2.5.0574
Ebert C, von Haken M, Meyer-Puttlitz B, Wiestler OD, Reifenberger 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(2):627–632. doi:10.1016/S0002-9440(10)65158-9
Ebner FH, Schittenhelm J, Roser F, Scheel-Walter H, Tatagiba M, Schuhmann MU (2012) Management of holocord pilocytic astrocytomas in children and adolescents: an update. Pediatr Neurosurg 48(3):133–140. doi:10.1159/000345593
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(4):1071–1077
Ellis JA, Canoll P, McCormick PC 2nd, Feldstein NA, Anderson RC, Angevine PD, Kaiser MG, McCormick PC, Bruce JN, Ogden AT (2012a) Platelet-derived growth factor receptor (PDGFR) expression in primary spinal cord gliomas. J Neuro Oncol 106(2):235–242. doi:10.1007/s11060-011-0666-6
Ellis JA, Castelli M, Bruce JN, Canoll P, Ogden AT (2012b) Retroviral delivery of platelet-derived growth factor to spinal cord progenitor cells drives the formation of intramedullary gliomas. Neurosurgery 70(1):198–204. doi:10.1227/NEU.0b013e31822ce963, discussion 204
Epstein NE (2005) A review of the risks and benefits of differing prophylaxis regimens for the treatment of deep venous thrombosis and pulmonary embolism in neurosurgery. Surg Neurol 64(4):295–301. doi:10.1016/j.surneu.2005.04.039, discussion 302
Epstein F, Epstein N (1981) Surgical management of holocord intramedullary spinal cord astrocytomas in children. J Neurosurg 54(6):829–832. doi:10.3171/jns.1981.54.6.0829
Epstein FJ, Farmer JP, Freed D (1992) Adult intramedullary astrocytomas of the spinal cord. J Neurosurg 77(3):355–359. doi:10.3171/jns.1992.77.3.0355
Epstein FJ, Farmer JP, Freed D (1993) Adult intramedullary spinal cord ependymomas: the result of surgery in 38 patients. J Neurosurg 79(2):204–209. doi:10.3171/jns.1993.79.2.0204
Evans DG, Huson SM, Donnai D, Neary W, Blair V, Teare D, Newton V, Strachan T, Ramsden R, Harris R (1992) A genetic study of type 2 neurofibromatosis in the United Kingdom. I. Prevalence, mutation rate, fitness, and confirmation of maternal transmission effect on severity. J Med Genet 29(12):841–846
Fakhreddine MH, Mahajan A, Penas-Prado M, Weinberg J, McCutcheon IE, Puduvalli V, Brown PD (2013) Treatment, prognostic factors, and outcomes in spinal cord astrocytomas. Neuro-Oncology 15(4):406–412. doi:10.1093/neuonc/nos309
Fine MJ, Kricheff II, Freed D, Epstein FJ (1995) Spinal cord ependymomas: MR imaging features. Radiology 197(3):655–658. doi:10.1148/radiology.197.3.7480734
Foreman NK, Love S, Thorne R (1996) Intracranial ependymomas: analysis of prognostic factors in a population-based series. Pediatr Neurosurg 24(3):119–125
Frappaz D, Ricci AC, Kohler R, Bret P, Mottolese C (1999) Diffuse brain stem tumor in an adolescent with multiple enchondromatosis (Ollier’s disease). Childs Nerv Syst 15(5):222–225. doi:10.1007/s003810050377
Garcia DM (1985) Primary spinal cord tumors treated with surgery and postoperative irradiation. Int J Radiat Oncol Biol Phys 11(11):1933–1939
Garcia C, Guttman DH (2014) Nf2/Merlin controls spinal cord neural progenitor function in a Rac1/ErbB2 dependent manner. PLoS One 9:e97320
Garrett PG, Simpson WJ (1983) Ependymomas: results of radiation treatment. Int J Radiat Oncol Biol Phys 9(8):1121–1124
Gilhuis HJ, van der Laak J, Wesseling P, Boerman RH, Beute G, Teepen JL, Grotenhuis JA, Kappelle AC (2004) Inverse correlation between genetic aberrations and malignancy grade in ependymal tumors: a paradox? J Neuro Oncol 66(1–2):111–116
Glasker S (2005) Central nervous system manifestations in VHL: genetics, pathology and clinical phenotypic features. Familial Cancer 4(1):37–42. doi:10.1007/s10689-004-5347-6
Glasker S, Bender BU, Apel TW, Natt E, van Velthoven V, Scheremet R, Zentner J, Neumann HP (1999) The impact of molecular genetic analysis of the VHL gene in patients with haemangioblastomas of the central nervous system. J Neurol Neurosurg Psychiatry 67(6):758–762
Glasker S, Bender BU, Apel TW, van Velthoven V, Mulligan LM, Zentner J, Neumann HP (2001) Reconsideration of biallelic inactivation of the VHL tumour suppressor gene in hemangioblastomas of the central nervous system. J Neurol Neurosurg Psychiatry 70(5):644–648
Glavac D, Neumann HP, Wittke C, Jaenig H, Masek O, Streicher T, Pausch F, Engelhardt D, Plate KH, Hofler H, Chen F, Zbar B, Brauch H (1996) Mutations in the VHL tumor suppressor gene and associated lesions in families with von Hippel-Lindau disease from central Europe. Hum Genet 98(3):271–280
Goh KY, Velasquez L, Epstein FJ (1997) Pediatric intramedullary spinal cord tumors: is surgery alone enough? Pediatr Neurosurg 27(1):34–39
Goh KYC, Muszynski CA, Teo JGC, Constantini S, Epstein FJ (2000) Excision of intramedullary tumours. In: Black PM, Kaye AH (eds) Operative neurosurgery. Churchill, London, pp 1947–1960
Grill J, Le Deley MC, Gambarelli D, Raquin MA, Couanet D, Pierre-Kahn A, Habrand JL, Doz F, Frappaz D, Gentet JC, Edan C, Chastagner P, Kalifa C (2001) Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology. J Clin Oncol 19(5):1288–1296
Guidetti B, Mercuri S, Vagnozzi R (1981) Long-term results of the surgical treatment of 129 intramedullary spinal gliomas. J Neurosurg 54(3):323–330. doi:10.3171/jns.1981.54.3.0323
Guss ZD, Moningi S, Jallo GI, Cohen KJ, Wharam MD, Terezakis SA (2013) Management of pediatric spinal cord astrocytomas: outcomes with adjuvant radiation. Int J Radiat Oncol Biol Phys 85(5):1307–1311. doi:10.1016/j.ijrobp.2012.11.022
Hanbali F, Fourney DR, Marmor E, Suki D, Rhines LD, Weinberg JS, McCutcheon IE, Suk I, Gokaslan ZL (2002) Spinal cord ependymoma: radical surgical resection and outcome. Neurosurgery 51(5):1162–1172, discussion 1172–1164
Hayashi Y, Nakada M, Mohri M, Murakami H, Kawahara N, Hamada J (2011) Ganglioglioma of the thoracolumbar spinal cord in a patient with neurofibromatosis type 1: a case report and literature review. Pediatr Neurosurg 47(3):210–213. doi:10.1159/000331569
Helseth A, Mork SJ (1989) Primary intraspinal neoplasms in Norway, 1955 to 1986. A population-based survey of 467 patients. J Neurosurg 71(6):842–845. doi:10.3171/jns.1989.71.6.0842
Hoshimaru M, Koyama T, Hashimoto N, Kikuchi H (1999) Results of microsurgical treatment for intramedullary spinal cord ependymomas: analysis of 36 cases. Neurosurgery 44(2):264–269
Houten JK, Cooper PR (2000) Spinal cord astrocytomas: presentation, management and outcome. J Neuro Oncol 47(3):219–224
Houten JK, Weiner HL (2000) Pediatric intramedullary spinal cord tumors: special considerations. J Neuro Oncol 47(3):225–230
Huang B, Starostik P, Schraut H, Krauss J, Sorensen N, Roggendorf W (2003) Human ependymomas reveal frequent deletions on chromosomes 6 and 9. Acta Neuropathol 106(4):357–362. doi:10.1007/s00401-003-0739-5
Hulsebos TJ, Oskam NT, Bijleveld EH, Westerveld A, Hermsen MA, van den Ouweland AM, Hamel BC, Tijssen CC (1999) Evidence for an ependymoma tumour suppressor gene in chromosome region 22pter-22q11.2. Br J Cancer 81(7):1150–1154. doi:10.1038/sj.bjc.6690822
Hulshof MC, Menten J, Dito JJ, Dreissen JJ, van den Bergh R, Gonzalez Gonzalez D (1993) Treatment results in primary intraspinal gliomas. Radiother Oncol 29(3):294–300
Innocenzi G, Salvati M, Cervoni L, Delfini R, Cantore G (1997) Prognostic factors in intramedullary astrocytomas. Clin Neurol Neurosurg 99(1):1–5
Isaacson SR (2000) Radiation therapy and the management of intramedullary spinal cord tumors. J Neuro Oncol 47(3):231–238
Isik N, Balak N, Silav G, Elmaci I (2008) Pediatric intramedullary teratomas. Neuropediatrics 39(4):196–199. doi:10.1055/s-0028-1112116
Ito T, Sawakami K, Ishikawa S, Hirano T, Endo N, Kakita A, Takahashi H (2013) Progression of paralysis is the most useful factor for differentiating malignant from benign intramedullary tumors. Spinal Cord 51(4):319–321. doi:10.1038/sc.2012.152
Iwasaki Y, Hida K, Sawamura Y, Abe H (2000) Spinal intramedullary ependymomas: surgical results and immunohistochemical analysis of tumour proliferation activity. Br J Neurosurg 14(4):331–336
Jallo GI, Danish S, Velasquez L, Epstein F (2001) Intramedullary low-grade astrocytomas: long-term outcome following radical surgery. J Neuro Oncol 53(1):61–66
Jones SJ, Harrison R, Koh KF, Mendoza N, Crockard HA (1996) Motor evoked potential monitoring during spinal surgery: responses of distal limb muscles to transcranial cortical stimulation with pulse trains. Electroencephalogr Clin Neurophysiol 100(5):375–383
Jones DT, Kocialkowski S, Liu L, Pearson DM, Backlund LM, Ichimura K, Collins VP (2008) Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas. Cancer Res 68(21):8673–8677. doi:10.1158/0008-5472.CAN-08-2097
Karikari IO, Nimjee SM, Hodges TR, Cutrell E, Hughes BD, Powers CJ, Mehta AI, Hardin C, Bagley CA, Isaacs RE, Haglund MM, Friedman AH (2011) Impact of tumor histology on resectability and neurological outcome in primary intramedullary spinal cord tumors: a single-center experience with 102 patients. Neurosurgery 68(1):188–197. doi:10.1227/NEU.0b013e3181fe3794, discussion 197
Kim WY, Kaelin WG (2004) Role of VHL gene mutation in human cancer. J Clin Oncol 22(24):4991–5004. doi:10.1200/JCO.2004.05.061
Kim NR, Suh YL, Shin HJ (2009) Thoracic pediatric intramedullary schwannoma: report of a case. Pediatr Neurosurg 45(5):396–401. doi:10.1159/000260911
Kim DH, Kim JH, Choi SH, Sohn CH, Yun TJ, Kim CH, Chang KH (2014) Differentiation between intramedullary spinal ependymoma and astrocytoma: comparative MRI analysis. Clin Radiol 69(1):29–35. doi:10.1016/j.crad.2013.07.017
Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK (2002) The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 61(3):215–225, discussion 226–219
Kley N, Whaley J, Seizinger BR (1995) Neurofibromatosis type 2 and von Hippel-Lindau disease: from gene cloning to function. Glia 15(3):297–307. doi:10.1002/glia.440150310
Knafo S, Court C, Parker F (2014) Predicting sagittal deformity after surgery for intramedullary tumors. J Neurosurg Spine 21(3):342–347. doi:10.3171/2014.5.SPINE13886
Kopelson G, Linggood RM (1982) Intramedullary spinal cord astrocytoma versus glioblastoma: the prognostic importance of histologic grade. Cancer 50(4):732–735
Kothbauer KF (2007) Neurosurgical management of intramedullary spinal cord tumors in children. Pediatr Neurosurg 43(3):222–235. doi:10.1159/000098835
Kramer DL, Parmiter AH, Rorke LB, Sutton LN, Biegel JA (1998) Molecular cytogenetic studies of pediatric ependymomas. J Neuro Oncol 37(1):25–33
Lamszus K, Lachenmayer L, Heinemann U, Kluwe L, Finckh U, Hoppner W, Stavrou D, Fillbrandt R, Westphal M (2001) Molecular genetic alterations on chromosomes 11 and 22 in ependymomas. Int J Cancer 91(6):803–808
Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott IR, Miller DC (1993) Central nervous system gangliogliomas. Part 2: clinical outcome. J Neurosurg 79(6):867–873. doi:10.3171/jns.1993.79.6.0867
Lee M, Rezai AR, Freed D, Epstein FJ (1996) Intramedullary spinal cord tumors in neurofibromatosis. Neurosurgery 38(1):32–37
Lee M, Epstein FJ, Rezai AR, Zagzag D (1998) Nonneoplastic intramedullary spinal cord lesions mimicking tumors. Neurosurgery 43(4):788–794, discussion 794–785
Lee SH, Chung CK, Kim CH, Yoon SH, Hyun SJ, Kim KJ, Kim ES, Eoh W, Kim HJ (2013) Long-term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: a retrospective multicenter study by the Korea Spinal Oncology Research Group. Neuro-Oncology 15(7):921–929. doi:10.1093/neuonc/not038
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(6):1397–1403
Liu W, James CD, Frederick L, Alderete BE, Jenkins RB (1997) PTEN/MMAC1 mutations and EGFR amplification in glioblastomas. Cancer Res 57(23):5254–5257
Liu X, Tian W, Kolar B, Hu R, Huang Y, Huang J, Ekholm S (2014) Advanced MR diffusion tensor imaging and perfusion weighted imaging of intramedullary tumors and tumor like lesions in the cervicomedullary junction region and the cervical spinal cord. J Neuro Oncol 116(3):559–566. doi:10.1007/s11060-013-1323-z
Louis DNOH, Wiestler OD, Cavenee WK (2007) WHO classification of tumors of the central nervous system. International Agency for Research on Cancer, Lyon
Madhukar M, Maller VG, Choudhary AK, Iantosca MR, Specht CS, Dias MS (2013) Primary intramedullary spinal cord germinoma. J Neurosurg Pediatr 11(5):605–609. doi:10.3171/2013.1.PEDS12402
Maiuri F, Iaconetta G, Gallicchio B, Stella L (2000) Intraoperative sonography for spinal tumors. Correlations with MR findings and surgery. J Neurosurg Sci 44(3):115–122
Martuza RL, Eldridge R (1988) Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med 318(11):684–688. doi:10.1056/NEJM198803173181106
Massimino M, Spreafico F, Cefalo G, Riccardi R, Tesoro-Tess JD, Gandola L, Riva D, Ruggiero A, Valentini L, Mazza E, Genitori L, Di Rocco C, Navarria P, Casanova M, Ferrari A, Luksch R, Terenziani M, Balestrini MR, Colosimo C, Fossati-Bellani F (2002) High response rate to cisplatin/etoposide regimen in childhood low-grade glioma. J Clin Oncol 20(20):4209–4216
Mautner VF, Tatagiba M, Guthoff R, Samii M, Pulst SM (1993) Neurofibromatosis 2 in the pediatric age group. Neurosurgery 33(1):92–96
McCormick PC, Stein BM (1990) Intramedullary tumors in adults. Neurosurg Clin N Am 1(3):609–630
McCormick PC, Post KD, Stein BM (1990a) Intradural extramedullary tumors in adults. Neurosurg Clin N Am 1(3):591–608
McCormick PC, Torres R, Post KD, Stein BM (1990b) Intramedullary ependymoma of the spinal cord. J Neurosurg 72(4):523–532. doi:10.3171/jns.1990.72.4.0523
McGirt MJ, Chaichana KL, Atiba A, Attenello F, Yao KC, Jallo GI (2008a) Resection of intramedullary spinal cord tumors in children: assessment of long-term motor and sensory deficits. J Neurosurg Pediatr 1(1):63–67. doi:10.3171/PED-08/01/063
McGirt MJ, Chaichana KL, Atiba A, Bydon A, Witham TF, Yao KC, Jallo GI (2008b) Incidence of spinal deformity after resection of intramedullary spinal cord tumors in children who underwent laminectomy compared with laminoplasty. J Neurosurg Pediatr 1(1):57–62. doi:10.3171/PED-08/01/057
McGirt MJ, Chaichana KL, Attenello F, Witham T, Bydon A, Yao KC, Jallo GI (2008c) Spinal deformity after resection of cervical intramedullary spinal cord tumors in children. Childs Nerv Syst 24(6):735–739. doi:10.1007/s00381-007-0513-4
McGirt MJ, Goldstein IM, Chaichana KL, Tobias ME, Kothbauer KF, Jallo GI (2008d) Extent of surgical resection of malignant astrocytomas of the spinal cord: outcome analysis of 35 patients. Neurosurgery 63(1):55–60. doi:10.1227/01.NEU.0000335070.37943.09, discussion 60–51
Mellon CD, Carter JE, Owen DB (1988) Ollier’s disease and Maffucci’s syndrome: distinct entities or a continuum. Case report: enchondromatosis complicated by an intracranial glioma. J Neurol 235(6):376–378
Miller DC (2000) Surgical pathology of intramedullary spinal cord neoplasms. J Neuro Oncol 47(3):189–194
Miller DJ, McCutcheon IE (2000) Hemangioblastomas and other uncommon intramedullary tumors. J Neuro Oncol 47(3):253–270
Minehan KJ, Shaw EG, Scheithauer BW, Davis DL, Onofrio BM (1995) Spinal cord astrocytoma: pathological and treatment considerations. J Neurosurg 83(4):590–595. doi:10.3171/jns.1995.83.4.0590
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(11):828–832
Mork SJ, Loken AC (1977) Ependymoma: a follow-up study of 101 cases. Cancer 40(2):907–915
Morota N, Deletis V, Constantini S, Kofler M, Cohen H, Epstein FJ (1997) The role of motor evoked potentials during surgery for intramedullary spinal cord tumors. Neurosurgery 41(6):1327–1336
Mulvihill JJ, Parry DM, Sherman JL, Pikus A, Kaiser-Kupfer MI, Eldridge R (1990) NIH conference. Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update. Ann Intern Med 113(1):39–52
Nash CL Jr, Lorig RA, Schatzinger LA, Brown RH (1977) Spinal cord monitoring during operative treatment of the spine. Clin Orthop Relat Res 126:100–105
Nazar GB, Hoffman HJ, Becker LE, Jenkin D, Humphreys RP, Hendrick EB (1990) Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg 72(3):408–417. doi:10.3171/jns.1990.72.3.0408
Neder L, Colli BO, Machado HR, Carlotti CG Jr, Santos AC, Chimelli L (2004) MIB-1 labeling index in astrocytic tumors – a clinicopathologic study. Clin Neuropathol 23(6):262–270
Neumann HP, Lips CJ, Hsia YE, Zbar B (1995) Von Hippel-Lindau syndrome. Brain Pathol 5(2):181–193
O’Sullivan C, Jenkin RD, Doherty MA, Hoffman HJ, Greenberg ML (1994) Spinal cord tumors in children: long-term results of combined surgical and radiation treatment. J Neurosurg 81(4):507–512. doi:10.3171/jns.1994.81.4.0507
Osborn A (1994) Diagnostic neuroradiology. Mosby, St. Louis
Parsa AT, McCormick PC (2005) Spinal axis tumors. In: Berger M, Prados M (eds) Textbook of neuro-oncology. Elsevier Saunders, Philadelphia, pp 476–484
Parsa AT, Lee J, Parney IF, Weinstein P, McCormick PC, Ames C (2004) Spinal cord and intradural-extraparenchymal spinal tumors: current best care practices and strategies. J Neuro Oncol 69(1–3):291–318
Parsons R (2004) Human cancer, PTEN and the PI-3 kinase pathway. Semin Cell Dev Biol 15(2):171–176
Pechstein U, Cedzich C, Nadstawek J, Schramm J (1996) Transcranial high-frequency repetitive electrical stimulation for recording myogenic motor evoked potentials with the patient under general anesthesia. Neurosurgery 39(2):335–343, discussion 343–334
Peker S, Ozgen S, Ozek MM, Pamir MN (2004) Surgical treatment of intramedullary spinal cord ependymomas: can outcome be predicted by tumor parameters? J Spinal Disord Tech 17(6):516–521
Perilongo G, Massimino M, Sotti G, Belfontali T, Masiero L, Rigobello L, Garre L, Carli M, Lombardi F, Solero C, Sainati L, Canale V, del Prever AB, Giangaspero F, Andreussi L, Mazza C, Madon E (1997) Analyses of prognostic factors in a retrospective review of 92 children with ependymoma: Italian Pediatric Neuro-oncology Group. Med Pediatr Oncol 29(2):79–85
Pollack IF, Gerszten PC, Martinez AJ, Lo KH, Shultz B, Albright AL, Janosky J, Deutsch M (1995) Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37(4):655–666, discussion 666–657
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 Neuro Oncol 32(3):235–241
Quinones-Hinojosa A, Lyon R, Zada G, Lamborn KR, Gupta N, Parsa AT, McDermott MW, Weinstein PR (2005) Changes in transcranial motor evoked potentials during intramedullary spinal cord tumor resection correlate with postoperative motor function. Neurosurgery 56(5):982–993, discussion 982–993
Raghavendra BN, Epstein FJ, McCleary L (1984) Intramedullary spinal cord tumors in children: localization by intraoperative sonography. AJNR Am J Neuroradiol 5(4):395–397
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(5):555–560. doi:10.3171/jns.1976.45.5.0555
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. Gene Chromosome Cancer 5(4):348–356
Rawlings CE 3rd, Giangaspero F, Burger PC, Bullard DE (1988) Ependymomas: a clinicopathologic study. Surg Neurol 29(4):271–281
Reimer R, Onofrio BM (1985) Astrocytomas of the spinal cord in children and adolescents. J Neurosurg 63(5):669–675. doi:10.3171/jns.1985.63.5.0669
Robertson PL, Allen JC, Abbott IR, Miller DC, Fidel J, Epstein FJ (1994) Cervicomedullary tumors in children: a distinct subset of brainstem gliomas. Neurology 44(10):1798–1803
Rodriguez HA, Berthrong M (1966) Multiple primary intracranial tumors in von Recklinghausen’s neurofibromatosis. Arch Neurol 14(5):467–475
Roonprapunt C, Silvera VM, Setton A, Freed D, Epstein FJ, Jallo GI (2001) Surgical management of isolated hemangioblastomas of the spinal cord. Neurosurgery 49(2):321–327, discussion 327–328
Rossitch E Jr, Zeidman SM, Burger PC, Curnes JT, Harsh C, Anscher M, Oakes WJ (1990) Clinical and pathological analysis of spinal cord astrocytomas in children. Neurosurgery 27(2):193–196
Rouleau GA, Wertelecki W, Haines JL, Hobbs WJ, Trofatter JA, Seizinger BR, Martuza RL, Superneau DW, Conneally PM, Gusella JF (1987) Genetic linkage of bilateral acoustic neurofibromatosis to a DNA marker on chromosome 22. Nature 329(6136):246–248. doi:10.1038/329246a0
Rouleau GA, Merel P, Lutchman M, Sanson M, Zucman J, Marineau C, Hoang-Xuan K, Demczuk S, Desmaze C, Plougastel B et al (1993) Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature 363(6429):515–521. doi:10.1038/363515a0
Rousseau P, Habrand JL, Sarrazin D, Kalifa C, Terrier-Lacombe MJ, Rekacewicz C, Rey A (1994) Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28(2):381–386
Rubio MP, Correa KM, Ramesh V, MacCollin MM, Jacoby LB, von Deimling A, Gusella JF, Louis DN (1994) Analysis of the neurofibromatosis 2 gene in human ependymomas and astrocytomas. Cancer Res 54(1):45–47
Russell DSRL (1989) Pathology of tumors of the nervous system. Williams & Wilkins, Baltimore
Samii M, Klekamp J (1994) Surgical results of 100 intramedullary tumors in relation to accompanying syringomyelia. Neurosurgery 35(5):865–873, discussion 873
Sandalcioglu IE, Gasser T, Asgari S, Lazorisak A, Engelhorn T, Egelhof T, Stolke D, Wiedemayer H (2005) Functional outcome after surgical treatment of intramedullary spinal cord tumors: experience with 78 patients. Spinal Cord 43(1):34–41. doi:10.1038/sj.sc.3101668
Sandler HM, Papadopoulos SM, Thornton AF Jr, Ross DA (1992) Spinal cord astrocytomas: results of therapy. Neurosurgery 30(4):490–493
Santi M, Quezado M, Ronchetti R, Rushing EJ (2005) Analysis of chromosome 7 in adult and pediatric ependymomas using chromogenic in situ hybridization. J Neuro Oncol 72(1):25–28. doi:10.1007/s11060-004-3117-9
Scheil S, Bruderlein S, Eicker M, Herms J, Herold-Mende C, Steiner HH, Barth TF, Moller P (2001) Low frequency of chromosomal imbalances in anaplastic ependymomas as detected by comparative genomic hybridization. Brain Pathol 11(2):133–143
Schiffer D, Giordana MT (1998) Prognosis of ependymoma. Childs Nerv Syst 14(8):357–361. doi:10.1007/s003810050244
Schneider C, Hidalgo ET, Schmitt-Mechelke T, Kothbauer KF (2014) Quality of life after surgical treatment of primary intramedullary spinal cord tumors in children. J Neurosurg Pediatr 13(2):170–177. doi:10.3171/2013.11.PEDS13346
Schwartz TH, McCormick PC (2000) Intramedullary ependymomas: clinical presentation, surgical treatment strategies and prognosis. J Neuro Oncol 47(3):211–218
Shaw EG, Evans RG, Scheithauer BW, Ilstrup DM, Earle JD (1986) Radiotherapeutic management of adult intraspinal ependymomas. Int J Radiat Oncol Biol Phys 12(3):323–327
Shrivastava RK, Epstein FJ, Perin NI, Post KD, Jallo GI (2005) Intramedullary spinal cord tumors in patients older than 50 years of age: management and outcome analysis. J Neurosurg Spine 2(3):249–255. doi:10.3171/spi.2005.2.3.0249
Singh A, Chand K, Singh H, Sarkar C, Sharma MC (2007) Atypical neurocytoma of the spinal cord in a young child. Childs Nerv Syst 23(2):207–211. doi:10.1007/s00381-006-0141-4
Smith SF, Simpson JM, Sekhon LH (2004) Prophylaxis for deep venous thrombosis in neurosurgical oncology: review of 2779 admissions over a 9-year period. Neurosurg Focus 17(4):E4
Sonneland PR, Scheithauer BW, Onofrio BM (1985) Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer 56(4):883–893
Souweidane MM, Bouffet E, Finlay J (1998) The role of chemotherapy in newly diagnosed ependymoma of childhood. Pediatr Neurosurg 28(5):273–278
Steinbok P, Cochrane DD, Poskitt K (1992) Intramedullary spinal cord tumors in children. Neurosurg Clin N Am 3(4):931–945
Sun B, Wang C, Wang J, Liu A (2003) MRI features of intramedullary spinal cord ependymomas. J Neuroimaging 13(4):346–351
Teo C, Nakaji P, Symons P, Tobias V, Cohn R, Smee R (2003) Ependymoma. Childs Nerv Syst 19(5–6):270–285. doi:10.1007/s00381-003-0753-x
Trofatter JA, MacCollin MM, Rutter JL, Murrell JR, Duyao MP, Parry DM, Eldridge R, Kley N, Menon AG, Pulaski K et al (1993) A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell 75(4):826
Valera ET, Serafini LN, Machado HR, Tone LG (2003) Complete surgical resection in children with low-grade astrocytomas after neoadjuvant chemotherapy. Childs Nerv Syst 19(2):86–90. doi:10.1007/s00381-002-0704-y
van Nielen KM, de Jong BM (1999) A case of Ollier’s disease associated with two intracerebral low-grade gliomas. Clin Neurol Neurosurg 101(2):106–110
von Deimling A, Louis DN, Wiestler OD (1995) Molecular pathways in the formation of gliomas. Glia 15(3):328–338. doi:10.1002/glia.440150312
von Haken MS, White EC, Daneshvar-Shyesther L, Sih S, Choi E, Kalra R, Cogen PH (1996) Molecular genetic analysis of chromosome arm 17p and chromosome arm 22q DNA sequences in sporadic pediatric ependymomas. Gene Chromosome Cancer 17(1):37–44. doi:10.1002/(SICI)1098-2264(199609)17:1<37::AID-GCC6>3.0.CO;2-3
Vortmeyer AO, Gnarra JR, Emmert-Buck MR, Katz D, Linehan WM, Oldfield EH, Zhuang Z (1997) von Hippel-Lindau gene deletion detected in the stromal cell component of a cerebellar hemangioblastoma associated with von Hippel-Lindau disease. Hum Pathol 28(5):540–543
Waha A, Koch A, Hartmann W, Mack H, Schramm J, Sorensen N, Berthold F, Wiestler OD, Pietsch T, Waha A (2004) Analysis of HIC-1 methylation and transcription in human ependymomas. Int J Cancer 110(4):542–549. doi:10.1002/ijc.20165
Ward BA, Gutmann DH (2005) Neurofibromatosis 1: from lab bench to clinic. Pediatr Neurol 32(4):221–228. doi:10.1016/j.pediatrneurol.2004.11.002
Weil RJ, Lonser RR, DeVroom HL, Wanebo JE, Oldfield EH (2003) Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 98(1):95–105. doi:10.3171/jns.2003.98.1.0095
Whitaker SJ, Bessell EM, Ashley SE, Bloom HJ, Bell BA, Brada M (1991) Postoperative radiotherapy in the management of spinal cord ependymoma. J Neurosurg 74(5):720–728. doi:10.3171/jns.1991.74.5.0720
Yagi T, Ohata K, Haque M, Hakuba A (1997) Intramedullary spinal cord tumour associated with neurofibromatosis type 1. Acta Neurochir (Wien) 139(11):1055–1060
Yamamoto Y, Raffel C (1999) Spinal extradural neoplasms and intradural neoplasms. In: Albright AL, Pollack IF, Adelson PD (eds) Principles and practice of pediatric neurosurgery. Thieme, New York, pp 685–696
Yao KC, McGirt MJ, Chaichana KL, Constantini S, Jallo GI (2007) Risk factors for progressive spinal deformity following resection of intramedullary spinal cord tumors in children: an analysis of 161 consecutive cases. J Neurosurg 107(6 Suppl):463–468. doi:10.3171/PED-07/12/463
Zuccaro G, Sosa F, Cuccia V, Lubieniecky F, Monges J (1999) Lateral ventricle tumors in children: a series of 54 cases. Childs Nerv Syst 15(11–12):774–785. doi:10.1007/s003810050470
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2017 Springer International Publishing
About this chapter
Cite this chapter
Breshears, J.D., Sun, P.P., Auguste, K.I. (2017). Intramedullary Spinal Cord Tumors. In: Gupta, N., Banerjee, A., Haas-Kogan, D. (eds) Pediatric CNS Tumors. Pediatric Oncology. Springer, Cham. https://doi.org/10.1007/978-3-319-30789-3_10
Download citation
DOI: https://doi.org/10.1007/978-3-319-30789-3_10
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-30787-9
Online ISBN: 978-3-319-30789-3
eBook Packages: MedicineMedicine (R0)