Primary spinal cord tumors account for 5% to 10% of all adult spinal tumors1 and 4.5% of primary central nervous system (CNS) tumors. Approximately 850 to 1700 new adult cases of primary CNS spinal cord tumors are diagnosed each year in the United States. Primary spinal cord tumors unlike intracranial tumors do not show an association between grade and age at diagnosis.
Because tumor origin varies by anatomic site in the spinal cord, primary spinal cord tumors are classified by anatomic sublocation as either intradural intramedullary, intradural extramedullary, or extradural. Intradural intramedullary spinal cord tumors constitute 20% to 30% of all primary spinal cord tumors; the remaining 70% to 80% are intradural extramedullary. Most (90%) intradural intramedullary tumors are either ependymomas (60%–70%) or astrocytomas (30%–40%). For the remaining 10%, hemangioblastomas account for 3% to 8%, of which 15% to 25% are associated with von Hippel-Lindau (VHL) disease. Another 2% of intradural intramedullary spinal cord tumors are metastatic. Intradural extramedullary spinal cord tumors predominantly comprise either meningiomas (30%) or peripheral nerve sheath tumors (30%). Overall, and including the lumbar cistern, the most common intradural extramedullary tumor types are meningiomas (24.4%), ependymomas (23.7%), and schwannomas (21.2%). Anatomically, the sites of primary spinal cord tumors are the spinal cord (70.5%), spinal meninges (24.2%), and cauda equina (5.3%).2
The clinical presentation of primary spinal cord tumors is determined partially by the location (Tables 1 and 2). Pain is the most common presenting symptom regardless of location,1,3 and is manifested as back pain (27%), radicular pain (25%), or central pain (20%). Neurologic deficit is the second most common symptom and can occur in the form of motor (72%), sensory (39%), or sphincter disturbance (15%).3 Intramedullary tumors usually affect central gray matter and cause a syringomyelitic syndrome characterized by the disassociation between pain/temperature (loss) and tactile (preserved) sensations at affected levels, lower motor nerve dysfunction at the affected level, and upper motor nerve dysfunction caudal to the lesion. No symptoms are pathognomic for spinal cord tumors, although sacral sparing (i.e., maintenance of sensation in the sacral dermatomes) and upper motor deficits are common in intramedullary tumors, whereas a Brown-Séquard–type syndrome (hemi-cord dysfunction) is characteristic of extramedullary tumors. Extramedullary tumors mostly present clinically with pain and myelopathic symptoms, such as paraparesis. Schwannomas, in contrast to other tumors, are more prone to cause radicular symptoms.4
Intramedullary Spinal Cord Tumor: Topography
Engelhard HH, Villano JL, Porter KR. Clinical presentation, histology, and treatment in 430 patients with primary tumors of the spinal cord, spinal meninges, or cauda equina. J Neurosurg Spine 2010;13:67–77.
Raco A, Esposito V, Lenzi J. Long-term follow-up of intramedullary spinal cord tumors: a series of 202 cases. Neurosurgery 2005;56:972–981.
Setzer M, Murtagh RD, Murtagh FR. Diffusion tensor imaging tractography in patients with intramedullary tumors: comparison with intraoperative findings and value for prediction of tumor resectability. J Neurosurg Spine 2010;13:371–380.
Eroes CA, Zausinger S, Kreth FW. Intramedullary low grade astrocytoma and ependymoma. Surgical results and predicting factors for clinical outcome. Acta Neurochir (Wien) 2010;152:611–618.
Taricco MA, Guirado VM, Fontes RB. Surgical treatment of primary intramedullary spinal cord tumors in adult patients. Arq Neuropsiquiatr 2008;66:59–63.
Yasargil MG, Tranmer BI, Adamson TE. Unilateral partial hemi-laminectomy for the removal of extra- and intramedullary tumours and AVMs. Adv Tech Stand Neurosurg 1991;18:113–132.
Gerszten PC, Burton SA, Ozhasoglu C. Radiosurgery for benign intradural spinal tumors. Neurosurgery 2008;62:887–895; discussion 895–896.
Amirjamshidi A, Abbassioun K. Radiation-induced tumors of the central nervous system occurring in childhood and adolescence. Four unusual lesions in three patients and a review of the literature. Childs Nerv Syst 2000;16:390–397.
Evans DG, Birch JM, Ramsden RT. Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes. J Med Genet 2006;43:289–294.
Campello C, Le Floch A, Parker F. Neuroepithelial intramedullary spinal cord tumors in adults: study of 70 cases [abstract]. Presented at American Academy of Neurology Annual Meeting; April 25–May 2, 2009; Seattle, Washington.
Helseth A, Mork SJ. Primary intraspinal neoplasms in Norway, 1955 to 1986. A population-based survey of 467 patients. J Neurosurg 1989;71:842–845.
Heuer GG, Stiefel MF, Bailey RL. Acute paraparesis from hemorrhagic spinal ependymoma: diagnostic dilemma and surgical management. J Neurosurg Spine 2007;7:652–655.
Parsa AT, Chi JH, Acosta FL Jr. Intramedullary spinal cord tumors: molecular insights and surgical innovation. Clin Neuro-Clin Neurosurg 2005;52:76–84.
Peker S, Ozgen S, Ozek MM. Surgical treatment of intramedullary spinal cord ependymomas: can outcome be predicted by tumor parameters? J Spinal Disord Tech 2004;17:516–521.
Maesawa S, Takayasu M, Kajita Y. Effects of opening peritumoral syrinxes on motor evoked potentials during resection of an intramedullary spinal cord tumor—case report. Neurol Med Chir (Tokyo) 2004;44:258–262.
Tobias ME, McGirt MJ, Chaichana KL. Surgical management of long intramedullary spinal cord tumors. Childs Nerv Syst 2008;24:219–223.
Hanbali F, Fourney DR, Marmor E. Spinal cord ependymoma: radical surgical resection and outcome. Neurosurgery 2002;51:1162–1172; discussion 1172–1174.
Volpp PB, Han K, Kagan AR. Outcomes in treatment for intradural spinal cord ependymomas. Int J Radiat Oncol Biol Phys 2007;69:1199–1204.
Cooper PR, Epstein F. Radical resection of intramedullary spinal cord tumors in adults. Recent experience in 29 patients. J Neurosurg 1985;63:492–499.
Abdel-Wahab M, Etuk B, Palermo J. Spinal cord gliomas: a multi-institutional retrospective analysis. Int J Radiat Oncol Biol Phys 2006;64:1060–1071.
Chamberlain MC, Johnston SK. Temozolomide for recurrent intracranial supratentorial platinum-refractory ependymoma. Cancer 2009;115:4775–4782.
Linstadt DE, Wara WM, Leibel SA. Postoperative radiotherapy of primary spinal cord tumors. Int J Radiat Oncol Biol Phys 1989;16:1397–1403.
Waldron JN, Laperriere NJ, Jaakkimainen L. Spinal cord ependymomas: a retrospective analysis of 59 cases. Int J Radiat Oncol Biol Phys 1993;27:223–229.
Cervoni L, Celli P, Fortuna A. Recurrence of spinal ependymoma. Risk factors and long-term survival. Spine (Phila Pa 1976) 1994;19:2838–2841.
Sgouros S, Malluci CL, Jackowski A. Spinal ependymomas—the value of postoperative radiotherapy for residual disease control. Br J Neurosurg 1996;10:559–566.
Yagi T, Ohata K, Haque M. Intramedullary spinal cord tumour associated with neurofibromatosis type 1. Acta Neurochir (Wien) 1997;139:1055–1060.
Kaley T, Abrey L. Temozolomide for spinal cord high-grade gliomas [abstract]. Presented at American Academy of Neurology Annual Meeting; April 25–May 2, 2009; Seattle, Washington.
Allen JC, Aviner S, Yates AJ. 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 1998;88:215–220.
Lefkowitz IB, Packer RJ, Sutton LN. Results of the treatment of children with recurrent gliomas with lomustine and vincristine. Cancer 1988;61:896–902.
Lowis SP, Pizer BL, Coakham H. Chemotherapy for spinal cord astrocytoma: can natural history be modified? Childs Nerv Syst 1998;14:317–321.
Packer RJ, Lange B, Ater J. Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 1993;11:850–856.
Rodrigues GB, Waldron JN, Wong CS. A retrospective analysis of 52 cases of spinal cord glioma managed with radiation therapy. Int J Radiat Oncol Biol Phys 2000;48:837–842.
Minehan KJ, Brown PD, Scheithauer BW. Prognosis and treatment of spinal cord astrocytoma. Int J Radiat Oncol Biol Phys 2009;73:727–733.
Murota T, Symon L. Surgical management of hemangioblastoma of the spinal cord: a report of 18 cases. Neurosurgery 1989;25:699–707; discussion 708.
Irie K, Kuyama H, Nagao S. Spinal cord hemangioblastoma presenting with subarachnoid hemorrhage. Neurol Med Chir (Tokyo) 1998;38:355–358.
Kormos RL, Tucker WS, Bilbao JM. Subarachnoid hemorrhage due to a spinal cord hemangioblastoma: case report. Neurosurgery 1980;6:657–660.
Minami M, Hanakita J, Suwa H. Cervical hemangioblastoma with a past history of subarachnoid hemorrhage. Surg Neurol 1998;49:278–281.
Yu JS, Short MP, Schumacher J. Intramedullary hemorrhage in spinal cord hemangioblastoma. Report of two cases. J Neurosurg 1994;81:937–940.
Eskridge JM, McAuliffe W, Harris B. Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol 1996;17:525–531.
Standard SC, Ahuja A, Livingston K. Endovascular embolization and surgical excision for the treatment of cerebellar and brain stem hemangioblastomas. Surg Neurol 1994;41:405–410.
Tampieri D, Leblanc R, TerBrugge K. Preoperative embolization of brain and spinal hemangioblastomas. Neurosurgery 1993;33:502–505; discussion 505.
Vazquez-Anon V, Botella C, Beltran A. Preoperative embolization of solid cervicomedullary junction hemangioblastomas: report of two cases. Neuroradiology 1997;39:86–89.
Wanebo JE, Lonser RR, Glenn GM. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 2003;98:82–94.
Ryu SI, Kim DH, Chang SD. Stereotactic radiosurgery for hemangiomas and ependymomas of the spinal cord. Neurosurg Focus 2003;15:E10.
Schuch G, de Wit M, Holtje J. Case 2. Hemangioblastomas: diagnosis of von Hippel-Lindau disease and antiangiogenic treatment with SU5416. J Clin Oncol 2005;23:3624–3626.
Lu DC, Lawton MT. Clinical presentation and surgical management of intramedullary spinal cord cavernous malformations. Neurosurgical Focus 2010;29:E12.
Kuhn J, Brummendorf TH, Brassat U. Novel KRIT1 mutation and no molecular evidence of anticipation in a family with cerebral and spinal cavernous malformations. Eur Neurol 2009;61:154–158.
Lee YW, Lee ST, Cha JG. A novel KRIT1 gene mutation in a patient with cerebral and multiple spinal cavernous malformations. Ann Clin Lab Sci 2010;40:290–294.
Zevgaridis D, Medele RJ, Hamburger C. Cavernous haemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien) 1999;141:237–245.
Bian LG, Bertalanffy H, Sun QF. Intramedullary cavernous malformations: clinical features and surgical technique via hemi-laminectomy. Clin Neurol Neurosurg 2009;111:511–517.
Chi JH, Parsa AT. Intramedullary spinal cord metastasis: clinical management and surgical considerations. Neurosurg Clin N Am 2006;17:45–50.
Gasser T, Sandalcioglu IE, El Hamalawi B. Surgical treat-Surgical treatment of intramedullary spinal cord metastases of systemic cancer: functional outcome and prognosis. J Neurooncol 2005;73:163–168.
Kalayci M, Cagavi F, Gul S. Intramedullary spinal cord metastases: diagnosis and treatment—an illustrated review. Acta Neurochir (Wien) 2004;146:1347–1354; discussion 1354.
Sandalcioglu IE, Gasser T, Asgari S. Functional outcome after surgical treatment of intramedullary spinal cord tumors: experience with 78 patients. Spinal Cord 2005;43:34–41.
Aryan HE, Farin A, Nakaji P. Intramedullary spinal cord metastasis of lung adenocarcinoma presenting as Brown-Sequard syndrome. Surg Neurol 2004;61:72–76.
Sharma MC, Jain D, Sarkar C. Spinal teratomas: a clinico-pathological study of 27 patients. Acta Neurochir (Wien) 2009;151:245–252; discussion 252.
Biswas A, Puri T, Goyal S. Spinal intradural primary germ cell tumour--review of literature and case report. Acta Neurochir (Wien) 2009;151:277–284.
Nakamura M, Ishii K, Watanabe K. Long-term surgical outcomes for myxopapillary ependymomas of the cauda equina. Spine (Phila Pa 1976) 2009;34:E756–760.
Meneses MS, Leal AG, Periotto LB. Primary fi lum ter-Primary filum terminale ependymoma: a series of 16 cases. Arq Neuropsiquiatr 2008;66:529–533.
Lin YH, Huang CI, Wong TT. Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy. J Neurooncol 2005;71:205–210.
Akyurek S, Chang EL, Yu TK. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neurooncol 2006;80:177–183.
Jinnai T, Koyama T. Clinical characteristics of spinal nerve sheath tumors: analysis of 149 cases. Neurosurgery 2005;56:510–515.
Conti P, Pansini G, Mouchaty H. Spinal neurinomas: retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature. Surg Neurol 2004;61:34–43; discussion 44.
Houshmandi SS, Emnett RJ, Giovannini M. The neurofibromatosis 2 protein, merlin, regulates glial cell growth in an ErbB2- and Src-dependent manner. Mol Cell Biol 2009;29:1472–1486.
Carney JA. Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol 1990;14:206–222.
Abul-Kasim K, Thurnher MM, McKeever P. Intradural spinal tumors: current classification and MRI features. Neuroradiology 2008;50:301–314.
Friedman DP, Tartaglino LM, Flanders AE. Intradural schwannomas of the spine: MR findings with emphasis on contrast-enhancement characteristics. AJR Am J Roentgenol 1992;158:1347–1350.
Parmar HA, Ibrahim M, Castillo M. Pictorial essay: diverse imaging features of spinal schwannomas. J Comput Assist Tomogr 2007;31:329–334.
Singrakhia MD, Parmar H, Maheshwari M. Cervical schwannoma presenting as an expansile vertebral body lesion: report of two cases with a technical note on the surgical management. Surg Neurol 2006;66:192–196; discussion 196.
Dodd RL, Ryu MR, Kamnerdsupaphon P. CyberKnife radiosurgery for benign intradural extramedullary spinal tumors. Neurosurgery 2006;58:674–685.
Parsa AT, Lee J, Parney IF. Spinal cord and intradural-extraparenchymal spinal tumors: current best care practices and strategies. J Neurooncol 2004;69:291–318.
Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol 1988;45:575–578.
Packer RJ, Gutmann DH, Rubenstein A. Plexiform neu-Plexiform neurofibromas in NF1: toward biologic-based therapy. Neurology 2002;58:1461–1470.
Perry A, Giannini C, Raghavan R. Aggressive phenotypic and genotypic features in pediatric and NF2-associated meningiomas: a clinicopathologic study of 53 cases. J Neuropathol Exp Neurol 2001;60:994–1003.
Sacko O, Haegelen C, Mendes V. Spinal meningioma surgery in elderly patients with paraplegia or severe paraparesis: a multicenter study. Neurosurgery 2009;64:503–509; discussion 509–510.
Setzer M, Vatter H, Marquardt G. Management of spinal meningiomas: surgical results and a review of the literature. Neurosurg Focus 2007;23:E14.
Roux FX, Nataf F, Pinaudeau M. Intraspinal meningiomas: review of 54 cases with discussion of poor prognosis factors and modern therapeutic management. Surg Neurol 1996;46:458–463; discussion 463–464.
Chamberlain MC, Glantz MJ, Fadul CE. Recurrent meningioma: salvage therapy with long-acting somatostatin analogue. Neurology 2007;69:969–973.
Kaley T, Wen P, Karimi S. Phase II trial of sunitinib (SU011248) in patients with recurrent or inoperable meningioma [abstract]. Presented at Society for Neuro-Oncology Annual Meeting; November 20–23, 2008; Las Vegas, Nevada.
DeBoer R, Grimm SA, Chandler J. A phase II trial of PTK-787 (PTK/ZK) in recurrent or progressive meningiomas [abstract]. J Clin Oncol 2008;26(suppl 1):Abstract 2060.
Chamberlain MC, Glantz MJ. Interferon-alpha for recurrent World Health Organization grade 1 intracranial meningiomas. Cancer 2008;113:2146–2151.
Goodwin JW, Crowley J, Eyre HJ. A phase II evaluation of tamoxifen in unresectable or refractory meningiomas: a Southwest Oncology Group study. J Neurooncol 1993;15:75–77.
Grunberg SM, Weiss MH, Spitz IM. Treatment of unresect-Treatment of unresectable meningiomas with the antiprogesterone agent mifepristone. J Neurosurg 1991;74:861–866.
Mason WP, Gentili F, Macdonald DR. Stabilization of disease progression by hydroxyurea in patients with recurrent or unresectable meningioma. J Neurosurg 2002;97:341–346.