Spinal schwannoma epidemiology

Most intradural tumors are located within the intradural extramedullary compartment, and the most common tumors are spinal schwannomas and meningiomas. Other less common neoplasms include neurofibroma, solitary fibrous tumor,myxopapillary ependymoma, lymphoma, metastatic leptomeningeal disease, malignant peripheral nerve sheath tumor, and paraganglioma. 1).


Spinal schwannoma is most frequently seen in the cervical and lumbar regions, far more frequently than in the thoracic spine.

The incidence of spinal schwannoma is 0.3–0.5/100,000 individuals annually 2).

Its prevalence is similar in males and females, and it is usually diagnosed during the fourth and fifth decades of life 3).

Schwannomas have an incidence of 3% of all spinal tumors.

Most occur sporadically and are solitary, but they may also be associated with Neurofibromatosis type 2, but can occur with Neurofibromatosis type 1.

Spinal schwannoma constitutes approximately 25% of the intradural spinal tumors 4) 5) 6) 7) 8) 9) 10). 11) 12) 13) 14) 15).

Except in cases of neurofibromatosis, it is very rare for tumors of different pathological types to exist concurrently at the same spinal level, with only 9 cases reported to date, in which spinal meningioma was found with spinal schwannoma in 7 cases and with spinal neurofibroma in 2 cases 16).

In a paraspinal location, they are the commonest cause of intradural extramedullary tumors but may also be extradural or extramedullary 17).

Most are entirely intradural, but 8-32 % may be completely extradural 18) 19). 1-19 % are a combination, 6-23 % are dumbbell spinal schwannomas, and 1 % are intramedullary schwannomas.

The most common location of spinal schwannomas are the lumbar spine (48%) 20).

Schwannomas are frequently located in the extramedullary region, and may present as dumbbell shaped in 10-15% of cases. They may also be located at the intramedullary region. Ten percent of the tumors were in the extradural location, and 1% in the intradural intramedullary regional location.

Up to 2.7% of schwannomas are located in the retroperitoneal region 21).

Most arise from the dorsal root of spinal nerve (sensory) rootlets (75%). Paraspinal schwannomas involve the dorsal nerve roots, affecting people in the fourth and fifth decades of life 22).


1)
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2)
Seppala MT, Haltia MJ, Sankila RJ, Jaaskelainen JE, Heiskanen O. Long-term outcome after removal of spinal schwannoma: a clinicopathologi- cal study of 187 cases. J Neurosurg (1995) 83:621–6. doi:10.3171/jns.1995. 83.4.0621
3)
Jinnai T, Koyama T. Clinical characteristics of spinal nerve sheath tumors: analysis of 149 cases. Neurosurgery (2005) 56:510–5. doi:10.1227/01. NEU.0000153752.59565.BB
4)
Engelhard HH, Villano JL, Porter KR, et al. 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.
5)
Safavi-Abbasi S, Senoglu M, Theodore N, et al. Microsurgical management of spinal schwannomas: evaluation of 128 cases. J Neurosurg Spine. 2008;9:40–47.
6)
Holland K, Kaye AH. Spinal tumors in neurofibromatosis-2: management considerations - a review. J Clin Neurosci. 2009;16:169–177.
7)
Klekamp J, Samii M. Surgery of spinal nerve sheath tumors with special reference to neurofibromatosis. Neurosurgery. 1998;42:279–289.
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Celli P, Trillò G, Ferrante L. Spinal extradural schwannoma. J Neurosurg Spine. 2005;2:447–456.
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Jankowski R, Szmeja J, Nowak S, Sokół B, Blok T. Giant schwannoma of the lumbar spine: a case report. Neurol Neurochir Pol. 2010;44:91–95.
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Conti P, Pansini G, Mouchaty H, Capuano C, Conti R. Spinal neurinomas: retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature. Surg Neurol 2004; 61: 34-43.
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Seppälä MT, Haltia MJ, Sankila RJ, Jääskeläinen JE, Heiskanen O. Long-term outcome after removal of spinal schwannoma: a clinicopathological study of 187 cases. J Neurosurg. 1995 Oct;83(4):621-6. PubMed PMID: 7674010.
12)
De Verdelhan O, Haegelen C, Carsin-Nicol B, et al. MR imaging features of spinal schwannomas and meningiomas. J Neuroradiol. 2005;32:42–49.
13)
Ahn DK, Park HS, Choi DJ, Kim KS, Kim TW, Park SY. The surgical treatment for spinal intradural extramedullary tumors. Clin Orthop Surg. 2009;1:165–172.
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Sim JE, Noh SJ, Song YJ, Kim HD. Removal of intradural-extramedullary spinal cord tumors with unilateral limited laminectomy. J Korean Neurosurg Soc. 2008;43:232–236.
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McCormick PC, Post KD, Stein BM. Intradural extramedullary tumors in adults. Neurosurg Clin N Am. 1990;1:591–608.
16)
Zhan Z, Yan X, Nie W, Ding Y, Xu W, Huang H. Neurofibroma and Meningioma within a Single Dumbbell-Shaped Tumor at the Same Cervical Level without Neurofibromatosis: a Case Report and Literature Review. World Neurosurg. 2019 Jun 26. pii: S1878-8750(19)31788-7. doi: 10.1016/j.wneu.2019.06.142. [Epub ahead of print] PubMed PMID: 31254713.
17)
Wein S, Gaillard F. Intradural spinal tumours and their mimics: A review of radiographic features. Postgrad Med J. 2013;89(1054):457–69.
21)
Cury J, Coelho RF, Srougi M. Retroperitoneal schwannoma: Case series and literature review. Clin São Paulo Braz. 2007;62(3):359–62.
22)
Chamberlain MC, Tredway TL. Adult primary intradural spinal cord tumors: A review. Curr Neurol Neurosci Rep. 2011;11(3):320–8.
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