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
EngelhardHHVillanoJLPorterKR. Clinical presentation, histology, and treatment in 430 patients with primary tumors of the spinal cord, spinal meninges, or cauda equina. J Neurosurg Spine2010;13:67–77.
RacoAEspositoVLenziJ. Long-term follow-up of intramedullary spinal cord tumors: a series of 202 cases. Neurosurgery2005;56:972–981.
SetzerMMurtaghRDMurtaghFR. Diffusion tensor imaging tractography in patients with intramedullary tumors: comparison with intraoperative findings and value for prediction of tumor resectability. J Neurosurg Spine2010;13:371–380.
EroesCAZausingerSKrethFW. Intramedullary low grade astrocytoma and ependymoma. Surgical results and predicting factors for clinical outcome. Acta Neurochir (Wien)2010;152:611–618.
TariccoMAGuiradoVMFontesRB. Surgical treatment of primary intramedullary spinal cord tumors in adult patients. Arq Neuropsiquiatr2008;66:59–63.
YasargilMGTranmerBIAdamsonTE. Unilateral partial hemi-laminectomy for the removal of extra- and intramedullary tumours and AVMs. Adv Tech Stand Neurosurg1991;18:113–132.
AmirjamshidiAAbbassiounK. 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 Syst2000;16:390–397.
EvansDGBirchJMRamsdenRT. Malignant transformation and new primary tumours after therapeutic radiation for benign disease: substantial risks in certain tumour prone syndromes. J Med Genet2006;43:289–294.
CampelloCLe FlochAParkerF. Neuroepithelial intramedullary spinal cord tumors in adults: study of 70 cases [abstract]. Presented at American Academy of Neurology Annual Meeting; April25–May 22009; Seattle, Washington.
HelsethAMorkSJ. Primary intraspinal neoplasms in Norway, 1955 to 1986. A population-based survey of 467 patients. J Neurosurg1989;71:842–845.
HeuerGGStiefelMFBaileyRL. Acute paraparesis from hemorrhagic spinal ependymoma: diagnostic dilemma and surgical management. J Neurosurg Spine2007;7:652–655.
ParsaATChiJHAcostaFLJr. Intramedullary spinal cord tumors: molecular insights and surgical innovation. Clin Neuro-Clin Neurosurg2005;52:76–84.
PekerSOzgenSOzekMM. Surgical treatment of intramedullary spinal cord ependymomas: can outcome be predicted by tumor parameters?J Spinal Disord Tech2004;17:516–521.
MaesawaSTakayasuMKajitaY. 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.
HanbaliFFourneyDRMarmorE. Spinal cord ependymoma: radical surgical resection and outcome. Neurosurgery2002;51:1162–1172; discussion 1172–1174.
CooperPREpsteinF. Radical resection of intramedullary spinal cord tumors in adults. Recent experience in 29 patients. J Neurosurg1985;63:492–499.
Abdel-WahabMEtukBPalermoJ. Spinal cord gliomas: a multi-institutional retrospective analysis. Int J Radiat Oncol Biol Phys2006;64:1060–1071.
ChamberlainMCJohnstonSK. Temozolomide for recurrent intracranial supratentorial platinum-refractory ependymoma. Cancer2009;115:4775–4782.
WaldronJNLaperriereNJJaakkimainenL. Spinal cord ependymomas: a retrospective analysis of 59 cases. Int J Radiat Oncol Biol Phys1993;27:223–229.
SgourosSMalluciCLJackowskiA. Spinal ependymomas—the value of postoperative radiotherapy for residual disease control. Br J Neurosurg1996;10:559–566.
YagiTOhataKHaqueM. Intramedullary spinal cord tumour associated with neurofibromatosis type 1. Acta Neurochir (Wien)1997;139:1055–1060.
KaleyTAbreyL. Temozolomide for spinal cord high-grade gliomas [abstract]. Presented at American Academy of Neurology Annual Meeting; April25–May 22009; Seattle, Washington.
AllenJCAvinerSYatesAJ. 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 Neurosurg1998;88:215–220.
LefkowitzIBPackerRJSuttonLN. Results of the treatment of children with recurrent gliomas with lomustine and vincristine. Cancer1988;61:896–902.
PackerRJLangeBAterJ. Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol1993;11:850–856.
RodriguesGBWaldronJNWongCS. A retrospective analysis of 52 cases of spinal cord glioma managed with radiation therapy. Int J Radiat Oncol Biol Phys2000;48:837–842.
MurotaTSymonL. Surgical management of hemangioblastoma of the spinal cord: a report of 18 cases. Neurosurgery1989;25:699–707; discussion 708.
YuJSShortMPSchumacherJ. Intramedullary hemorrhage in spinal cord hemangioblastoma. Report of two cases. J Neurosurg1994;81:937–940.
EskridgeJMMcAuliffeWHarrisB. Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol1996;17:525–531.
StandardSCAhujaALivingstonK. Endovascular embolization and surgical excision for the treatment of cerebellar and brain stem hemangioblastomas. Surg Neurol1994;41:405–410.
TampieriDLeblancRTerBruggeK. Preoperative embolization of brain and spinal hemangioblastomas. Neurosurgery1993;33:502–505; discussion 505.
Vazquez-AnonVBotellaCBeltranA. Preoperative embolization of solid cervicomedullary junction hemangioblastomas: report of two cases. Neuroradiology1997;39:86–89.
WaneboJELonserRRGlennGM. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg2003;98:82–94.
SchuchGde WitMHoltjeJ. Case 2. Hemangioblastomas: diagnosis of von Hippel-Lindau disease and antiangiogenic treatment with SU5416. J Clin Oncol2005;23:3624–3626.
LuDCLawtonMT. Clinical presentation and surgical management of intramedullary spinal cord cavernous malformations. Neurosurgical Focus2010;29:E12.
KuhnJBrummendorfTHBrassatU. Novel KRIT1 mutation and no molecular evidence of anticipation in a family with cerebral and spinal cavernous malformations. Eur Neurol2009;61:154–158.
LeeYWLeeSTChaJG. A novel KRIT1 gene mutation in a patient with cerebral and multiple spinal cavernous malformations. Ann Clin Lab Sci2010;40:290–294.
ZevgaridisDMedeleRJHamburgerC. Cavernous haemangiomas of the spinal cord. A review of 117 cases. Acta Neurochir (Wien)1999;141:237–245.
BianLGBertalanffyHSunQF. Intramedullary cavernous malformations: clinical features and surgical technique via hemi-laminectomy. Clin Neurol Neurosurg2009;111:511–517.
ChiJHParsaAT. Intramedullary spinal cord metastasis: clinical management and surgical considerations. Neurosurg Clin N Am2006;17:45–50.
GasserTSandalciogluIEEl HamalawiB. Surgical treat-Surgical treatment of intramedullary spinal cord metastases of systemic cancer: functional outcome and prognosis. J Neurooncol2005;73:163–168.
KalayciMCagaviFGulS. Intramedullary spinal cord metastases: diagnosis and treatment—an illustrated review. Acta Neurochir (Wien)2004;146:1347–1354; discussion 1354.
SandalciogluIEGasserTAsgariS. Functional outcome after surgical treatment of intramedullary spinal cord tumors: experience with 78 patients. Spinal Cord2005;43:34–41.
AryanHEFarinANakajiP. Intramedullary spinal cord metastasis of lung adenocarcinoma presenting as Brown-Sequard syndrome. Surg Neurol2004;61:72–76.
SharmaMCJainDSarkarC. Spinal teratomas: a clinico-pathological study of 27 patients. Acta Neurochir (Wien)2009;151:245–252; discussion 252.
BiswasAPuriTGoyalS. Spinal intradural primary germ cell tumour--review of literature and case report. Acta Neurochir (Wien)2009;151:277–284.
NakamuraMIshiiKWatanabeK. Long-term surgical outcomes for myxopapillary ependymomas of the cauda equina. Spine (Phila Pa 1976)2009;34:E756–760.
MenesesMSLealAGPeriottoLB. Primary fi lum ter-Primary filum terminale ependymoma: a series of 16 cases. Arq Neuropsiquiatr2008;66:529–533.
LinYHHuangCIWongTT. Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy. J Neurooncol2005;71:205–210.
AkyurekSChangELYuTK. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neurooncol2006;80:177–183.
ContiPPansiniGMouchatyH. Spinal neurinomas: retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature. Surg Neurol2004;61:34–43; discussion 44.
HoushmandiSSEmnettRJGiovanniniM. The neurofibromatosis 2 protein, merlin, regulates glial cell growth in an ErbB2- and Src-dependent manner. Mol Cell Biol2009;29:1472–1486.
CarneyJA. Psammomatous melanotic schwannoma. A distinctive, heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol1990;14:206–222.
FriedmanDPTartaglinoLMFlandersAE. Intradural schwannomas of the spine: MR findings with emphasis on contrast-enhancement characteristics. AJR Am J Roentgenol1992;158:1347–1350.
SingrakhiaMDParmarHMaheshwariM. Cervical schwannoma presenting as an expansile vertebral body lesion: report of two cases with a technical note on the surgical management. Surg Neurol2006;66:192–196; discussion 196.
DoddRLRyuMRKamnerdsupaphonP. CyberKnife radiosurgery for benign intradural extramedullary spinal tumors. Neurosurgery2006;58:674–685.
ParsaATLeeJParneyIF. Spinal cord and intradural-extraparenchymal spinal tumors: current best care practices and strategies. J Neurooncol2004;69:291–318.
Neurofibromatosis. Conference statement. National Institutes of Health Consensus Development Conference. Arch Neurol1988;45:575–578.
PackerRJGutmannDHRubensteinA. Plexiform neu-Plexiform neurofibromas in NF1: toward biologic-based therapy. Neurology2002;58:1461–1470.
PerryAGianniniCRaghavanR. Aggressive phenotypic and genotypic features in pediatric and NF2-associated meningiomas: a clinicopathologic study of 53 cases. J Neuropathol Exp Neurol2001;60:994–1003.
SackoOHaegelenCMendesV. Spinal meningioma surgery in elderly patients with paraplegia or severe paraparesis: a multicenter study. Neurosurgery2009;64:503–509; discussion 509–510.
SetzerMVatterHMarquardtG. Management of spinal meningiomas: surgical results and a review of the literature. Neurosurg Focus2007;23:E14.
RouxFXNatafFPinaudeauM. Intraspinal meningiomas: review of 54 cases with discussion of poor prognosis factors and modern therapeutic management. Surg Neurol1996;46:458–463; discussion 463–464.
ChamberlainMCGlantzMJFadulCE. Recurrent meningioma: salvage therapy with long-acting somatostatin analogue. Neurology2007;69:969–973.
KaleyTWenPKarimiS. Phase II trial of sunitinib (SU011248) in patients with recurrent or inoperable meningioma [abstract]. Presented at Society for Neuro-Oncology Annual Meeting; November20–232008; Las Vegas, Nevada.
DeBoerRGrimmSAChandlerJ. A phase II trial of PTK-787 (PTK/ZK) in recurrent or progressive meningiomas [abstract]. J Clin Oncol2008;26(suppl 1):Abstract 2060.
ChamberlainMCGlantzMJ. Interferon-alpha for recurrent World Health Organization grade 1 intracranial meningiomas. Cancer2008;113:2146–2151.
GoodwinJWCrowleyJEyreHJ. A phase II evaluation of tamoxifen in unresectable or refractory meningiomas: a Southwest Oncology Group study. J Neurooncol1993;15:75–77.
GrunbergSMWeissMHSpitzIM. Treatment of unresect-Treatment of unresectable meningiomas with the antiprogesterone agent mifepristone. J Neurosurg1991;74:861–866.
MasonWPGentiliFMacdonaldDR. Stabilization of disease progression by hydroxyurea in patients with recurrent or unresectable meningioma. J Neurosurg2002;97:341–346.