Spinal cord tumors
- William C Welch, MD, FACS, FICS
William C Welch, MD, FACS, FICS
- Professor of Neurosurgery
- University of Pennsylvania School of Medicine
- Chief of Neurological Surgery
- The Pennsylvania Hospital
- David Schiff, MD
David Schiff, MD
- Professor of Neurology, Neurological Surgery, and Medicine
- University of Virginia School of Medicine
- Peter C Gerszten, MD, MPH, FACS
Peter C Gerszten, MD, MPH, FACS
- Professor of Neurosurgery and Radiation Oncology
- University of Pittsburgh Medical Center
- Section Editors
- Jay S Loeffler, MD
Jay S Loeffler, MD
- Section Editor — Neurooncology
- Professor of Radiation Oncology
- Harvard Medical School
- Patrick Y Wen, MD
Patrick Y Wen, MD
- Section Editor — Neurooncology
- Professor of Neurology
- Harvard Medical School
Spinal cord tumors can occur within or adjacent to the spinal cord. They are considered to be intraaxial in location and can be either primary or metastatic. Primary spinal cord tumors account for 2 to 4 percent of all primary central nervous system (CNS) tumors, one-third of which are located in the intramedullary compartment.
Spinal cord tumors can be classified according to their anatomic location (figure 1):
●Intramedullary – Intramedullary tumors arise within the spinal cord itself. Most primary intramedullary tumors are either ependymomas or astrocytomas. Metastases are being recognized with increasing frequency, primarily because of improvements in imaging modalities.
●Intradural-extramedullary – Tumors arising within the dura but outside the actual spinal cord are termed intradural-extramedullary. The most common tumors in this group are meningiomas and nerve sheath tumors. (See "Epidemiology, pathology, clinical features, and diagnosis of meningioma" and "Intradural nerve sheath tumors".)
●Extradural – Extradural tumors are usually metastatic and most often arise in the vertebral bodies. Metastatic lesions can cause spinal cord compression either by epidural growth that results in extrinsic spinal cord or cauda equina compression or less frequently by intradural invasion. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression, including cauda equina syndrome".)
- Duong LM, McCarthy BJ, McLendon RE, et al. Descriptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004-2007. Cancer 2012; 118:4220.
- Welch WC, Jacobs GB. Surgery for metastatic spinal disease. J Neurooncol 1995; 23:163.
- Welch WC, Erhard R, Clyde B, Jacobs GB. Systemic malignancy presenting as neck and shoulder pain. Arch Phys Med Rehabil 1994; 75:918.
- Black P. Spinal metastasis: current status and recommended guidelines for management. Neurosurgery 1979; 5:726.
- Posner JB. Neurologic Complications of Cancer, FA Davis, Philadelphia 1995.
- Sevick RJ, Wallace CJ. MR imaging of neoplasms of the lumbar spine. Magn Reson Imaging Clin N Am 1999; 7:539.
- Shrivastava RK, Epstein FJ, Perin NI, et al. Intramedullary spinal cord tumors in patients older than 50 years of age: management and outcome analysis. J Neurosurg Spine 2005; 2:249.
- Waldron JN, Laperriere NJ, Jaakkimainen L, et al. Spinal cord ependymomas: a retrospective analysis of 59 cases. Int J Radiat Oncol Biol Phys 1993; 27:223.
- Newton HB, Henson J, Walker RW. Extraneural metastases in ependymoma. J Neurooncol 1992; 14:135.
- Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. Classification of Tumours of the Nervous System, IARC Press, Lyon, France 2007.
- Intrinsic spinal cord tumors. In: Handbook of Clinical Neurology, Amsterdam 1997. Vol 24, p.497.
- McCormick PC, Torres R, Post KD, Stein BM. Intramedullary ependymoma of the spinal cord. J Neurosurg 1990; 72:523.
- Abdel-Wahab M, Etuk B, Palermo J, et al. Spinal cord gliomas: A multi-institutional retrospective analysis. Int J Radiat Oncol Biol Phys 2006; 64:1060.
- Lee SH, Chung CK, Kim CH, et al. 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 Oncol 2013; 15:921.
- Pica A, Miller R, Villà S, et al. The results of surgery, with or without radiotherapy, for primary spinal myxopapillary ependymoma: a retrospective study from the rare cancer network. Int J Radiat Oncol Biol Phys 2009; 74:1114.
- Akyurek S, Chang EL, Yu TK, et al. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neurooncol 2006; 80:177.
- Weber DC, Wang Y, Miller R, et al. Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro Oncol 2015; 17:588.
- NCCN guildines available at http://www.nccn.org/professionals/physician_gls/f_guidelines.asp#site (Accessed on March 23, 2015).
- Bagley CA, Wilson S, Kothbauer KF, et al. Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev 2009; 32:321.
- Ritter AM, Hess KR, McLendon RE, Langford LA. Ependymomas: MIB-1 proliferation index and survival. J Neurooncol 1998; 40:51.
- Minehan KJ, Brown PD, Scheithauer BW, et al. Prognosis and treatment of spinal cord astrocytoma. Int J Radiat Oncol Biol Phys 2009; 73:727.
- Rossitch E Jr, Zeidman SM, Burger PC, et al. Clinical and pathological analysis of spinal cord astrocytomas in children. Neurosurgery 1990; 27:193.
- Steinbok P. Spinal cord astrocytomas: long-term results comparing treatments in children. Childs Nerv Syst 1998; 14:1.
- Coakley KJ, Huston J 3rd, Scheithauer BW, et al. Pilocytic astrocytomas: well-demarcated magnetic resonance appearance despite frequent infiltration histologically. Mayo Clin Proc 1995; 70:747.
- WHO Classification of Tumours of the Central Nervous System, Revised 4th Edition, Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds), IARC, Lyon 2016.
- Babu R, Karikari IO, Owens TR, Bagley CA. Spinal cord astrocytomas: a modern 20-year experience at a single institution. Spine (Phila Pa 1976) 2014; 39:533.
- Lowis SP, Pizer BL, Coakham H, et al. Chemotherapy for spinal cord astrocytoma: can natural history be modified? Childs Nerv Syst 1998; 14:317.
- Doireau V, Grill J, Zerah M, et al. Chemotherapy for unresectable and recurrent intramedullary glial tumours in children. Brain Tumours Subcommittee of the French Society of Paediatric Oncology (SFOP). Br J Cancer 1999; 81:835.
- Allen JC, Aviner S, Yates AJ, et al. 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.
- Mora J, Cruz O, Gala S, Navarro R. Successful treatment of childhood intramedullary spinal cord astrocytomas with irinotecan and cisplatin. Neuro Oncol 2007; 9:39.
- Schiff D, O'Neill BP. Intramedullary spinal cord metastases: clinical features and treatment outcome. Neurology 1996; 47:906.
- Dam-Hieu P, Seizeur R, Mineo JF, et al. Retrospective study of 19 patients with intramedullary spinal cord metastasis. Clin Neurol Neurosurg 2009; 111:10.
- Lee SS, Kim MK, Sym SJ, et al. Intramedullary spinal cord metastases: a single-institution experience. J Neurooncol 2007; 84:85.
- Créange A, Zeller J, Rostaing-Rigattieri S, et al. Neurological complications of neurofibromatosis type 1 in adulthood. Brain 1999; 122 ( Pt 3):473.
- Antinheimo J, Sankila R, Carpén O, et al. Population-based analysis of sporadic and type 2 neurofibromatosis-associated meningiomas and schwannomas. Neurology 2000; 54:71.
- Dow G, Biggs N, Evans G, et al. Spinal tumors in neurofibromatosis type 2. Is emerging knowledge of genotype predictive of natural history? J Neurosurg Spine 2005; 2:574.
- Lee JW, Lee IS, Choi KU, et al. CT and MRI findings of calcified spinal meningiomas: correlation with pathological findings. Skeletal Radiol 2010; 39:345.
- Gerszten PC, Burton SA, Ozhasoglu C, et al. Radiosurgery for benign intradural spinal tumors. Neurosurgery 2008; 62:887.
- Sachdev S, Dodd RL, Chang SD, et al. Stereotactic radiosurgery yields long-term control for benign intradural, extramedullary spinal tumors. Neurosurgery 2011; 69:533.
- Stacchiotti S, Casali PG, Lo Vullo S, et al. Chordoma of the mobile spine and sacrum: a retrospective analysis of a series of patients surgically treated at two referral centers. Ann Surg Oncol 2010; 17:211.
- York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery 1999; 44:74.
- Osaka S, Kodoh O, Sugita H, et al. Clinical significance of a wide excision policy for sacrococcygeal chordoma. J Cancer Res Clin Oncol 2006; 132:213.
- Cheng EY, Ozerdemoglu RA, Transfeldt EE, Thompson RC Jr. Lumbosacral chordoma. Prognostic factors and treatment. Spine (Phila Pa 1976) 1999; 24:1639.
- Park L, Delaney TF, Liebsch NJ, et al. Sacral chordomas: Impact of high-dose proton/photon-beam radiation therapy combined with or without surgery for primary versus recurrent tumor. Int J Radiat Oncol Biol Phys 2006; 65:1514.
- DeLaney TF, Liebsch NJ, Pedlow FX, et al. Phase II study of high-dose photon/proton radiotherapy in the management of spine sarcomas. Int J Radiat Oncol Biol Phys 2009; 74:732.
- Henderson FC, McCool K, Seigle J, et al. Treatment of chordomas with CyberKnife: georgetown university experience and treatment recommendations. Neurosurgery 2009; 64:A44.
- Imai R, Kamada T, Tsuji H, et al. Effect of carbon ion radiotherapy for sacral chordoma: results of Phase I-II and Phase II clinical trials. Int J Radiat Oncol Biol Phys 2010; 77:1470.
- Indelicato DJ, Rotondo RL, Begosh-Mayne D, et al. A Prospective Outcomes Study of Proton Therapy for Chordomas and Chondrosarcomas of the Spine. Int J Radiat Oncol Biol Phys 2016; 95:297.
- Imai R, Kamada T, Araki N, Working Group for Bone and Soft Tissue Sarcomas. Carbon Ion Radiation Therapy for Unresectable Sacral Chordoma: An Analysis of 188 Cases. Int J Radiat Oncol Biol Phys 2016; 95:322.
- NCCN Clinical Practice Guidelines in Oncology. Soft Tissue Sarcoma. V.2.2010. http://www.nccn.org/professionals/physician_gls/PDF/sarcoma.pdf (Accessed on July 28, 2010).
- Stacchiotti S, Longhi A, Ferraresi V, et al. Phase II study of imatinib in advanced chordoma. J Clin Oncol 2012; 30:914.
- Casali PG, Messina A, Stacchiotti S, et al. Imatinib mesylate in chordoma. Cancer 2004; 101:2086.
- Stacchiotti S, Marrari A, Tamborini E, et al. Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol 2009; 20:1886.
- Ferraresi V, Nuzzo C, Zoccali C, et al. Chordoma: clinical characteristics, management and prognosis of a case series of 25 patients. BMC Cancer 2010; 10:22.
- Casali, PG, Stacchiotti, S, Grosso, F, et al. Adding cisplatin to imatinib re-establishes tumor response following secondary resistance to imatinib in advanced chorcoma (abstract 10038). J Clin Oncol 2007; 25:554s.
- Hindi N, Casali PG, Morosi C, et al. Imatinib in advanced chordoma: A retrospective case series analysis. Eur J Cancer 2015; 51:2609.
- George S, Merriam P, Maki RG, et al. Multicenter phase II trial of sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas. J Clin Oncol 2009; 27:3154.
- Bompas E, Le Cesne A, Tresch-Bruneel E, et al. Sorafenib in patients with locally advanced and metastatic chordomas: a phase II trial of the French Sarcoma Group (GSF/GETO). Ann Oncol 2015; 26:2168.
- Singhal N, Kotasek D, Parnis FX. Response to erlotinib in a patient with treatment refractory chordoma. Anticancer Drugs 2009; 20:953.
- Azzarelli A, Quagliuolo V, Cerasoli S, et al. Chordoma: natural history and treatment results in 33 cases. J Surg Oncol 1988; 37:185.
- Jacob HE. Chemotherapy for cranial base tumors. J Neurooncol 1994; 20:327.
- Hansen MF, Nellissery MJ, Bhatia P. Common mechanisms of osteosarcoma and Paget's disease. J Bone Miner Res 1999; 14 Suppl 2:39.
- Marshman LA, Pollock JR, King A, Chawda SJ. Primary extradural epithelioid leiomyosarcoma of the cervical spine: case report and literature review. Neurosurgery 2005; 57:E372; discussion E372.
- Kokubo Y, Uchida K, Kobayashi S, et al. Primary osteosarcoma of the thoracic spine: report of an unusual elderly patient with autopsy findings. Spinal Cord 2005; 43:508.
- Sansur CA, Pouratian N, Dumont AS, et al. Part II: spinal-cord neoplasms--primary tumours of the bony spine and adjacent soft tissues. Lancet Oncol 2007; 8:137.
- Pastushyn AI, Slin'ko EI, Mirzoyeva GM. Vertebral hemangiomas: diagnosis, management, natural history and clinicopathological correlates in 86 patients. Surg Neurol 1998; 50:535.
- Kransdorf MJ, Sweet DE. Aneurysmal bone cyst: concept, controversy, clinical presentation, and imaging. AJR Am J Roentgenol 1995; 164:573.
- Papagelopoulos PJ, Currier BL, Shaughnessy WJ, et al. Aneurysmal bone cyst of the spine. Management and outcome. Spine (Phila Pa 1976) 1998; 23:621.
- Physical examination
- INTRAMEDULLARY TUMORS
- - Ependymoma
- - Myxopapillary ependymoma
- - Anaplastic ependymoma
- INTRADURAL EXTRAMEDULLARY TUMORS
- Nerve sheath tumors
- EXTRADURAL PRIMARY TUMORS
- - Surgery and RT
- - Systemic therapy
- Plasmacytomas and multiple myeloma
- Langerhans cell histiocytosis (eosinophilic granuloma)
- Benign lesions
- SUMMARY AND RECOMMENDATIONS
- Primary intramedullary tumors
- Intradural extramedullary lesions
- Extradural tumors