Nerve sheath tumor
| Nerve sheath tumors | |
|---|---|
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| Illustration of Schwannoma (a.) and Neurofibroma (b.) configurations around nerves. | |
| Specialty | Neuro-oncology |
A nerve sheath tumor is a type of tumor of the nervous system (nervous system neoplasm) which is made up primarily of the myelin surrounding nerves. Nerve sheath tumors can be benign or malignant, and may affect both the peripheral and central nervous systems. There are three general types of nerve sheath tumors: schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors.[1]
Classification of nerve sheath tumors
[edit]Spinal nerve sheath tumors
[edit]Spinal nerve sheath tumors are typically intradural, meaning that they arise inside the dura mater surrounding the spinal cord (thecal sac), but may also be found in other areas of the spine.[2] Spinal nerve sheath tumors generally arise as single lesions.[1] Presence of multiple lesions is associated with genetic conditions including neurofibromatosis type 1, neurofibromatosis type 2, and Schwannomatosis.[1][3][4]
Most spinal schwannomas are intradural-extramedullary, growing inside the thecal sac, but outside the spinal cord itself.[5] Intradural-intramedullary schwannomas have also been reported, but are rare.[5]
Peripheral nerve sheath tumors
[edit]A peripheral nerve sheath tumor is a nerve sheath tumor in the peripheral nervous system. Benign peripheral nerve sheath tumors include schwannomas and neurofibromas. A malignant peripheral nerve sheath tumor is a cancerous peripheral nerve sheath tumor, which are frequently resistant to conventional treatments.
Symptoms
[edit]Causes
[edit]Mechanism
[edit]The primary Schwann cell differentiation and neoplastic proliferations are characteristics of peripheral nerve sheath tumors. For instance, the Schwann cell, which is the major neoplastic cell component of neurofibroma,[6] is cytologically distinguished by the expression of S-100 protein and wavy nuclear outlines.[7][8] A variety of peripheral nerve cells, including axons, perineurial cells, fibroblasts, and varying inflammatory components such as mast cells and lymphocytes, are also present in neurofibromas. A population of CD34-positive cells with an unknown histogenesis is also found.[9][10]
Diagnosis
[edit]Screening
[edit]Outcomes
[edit]Epidemiology
[edit]References
[edit]- ^ a b c Gui C, Canthiya L, Zadeh G, Suppiah S (October 2024). "Current state of spinal nerve sheath tumor management and future advances". Neuro-Oncology Advances. 6 (Suppl 3): iii83–iii93. doi:10.1093/noajnl/vdae067. PMC 11485951. PMID 39430389.
- ^ Major NM, Anderson MW, Helms CA, Kaplan PA, Dussault R (2020-01-01). "Chapter 13 - Spine". In Major NM, Anderson MW, Helms CA, Kaplan PA (eds.). Musculoskeletal MRI (Third ed.). St. Louis (MO): Elsevier. pp. 295–346. ISBN 978-0-323-41560-6. Retrieved 2024-12-12.
- ^ Conti P, Pansini G, Mouchaty H, Capuano C, Conti R (January 2004). "Spinal neurinomas: retrospective analysis and long-term outcome of 179 consecutively operated cases and review of the literature". Surgical Neurology. 61 (1): 34–43, discussion 44. doi:10.1016/S0090-3019(03)00537-8. PMID 14706374.
- ^ Jinnai T, Koyama T (March 2005). "Clinical characteristics of spinal nerve sheath tumors: analysis of 149 cases". Neurosurgery. 56 (3): 510–515. doi:10.1227/01.NEU.0000153752.59565.BB. PMID 15730576.
- ^ a b Swiatek VM, Stein KP, Cukaz HB, Rashidi A, Skalej M, Mawrin C, et al. (August 2021). "Spinal intramedullary schwannomas-report of a case and extensive review of the literature". Neurosurgical Review. 44 (4): 1833–1852. doi:10.1007/s10143-020-01357-5. PMC 8338859. PMID 32935226.
- ^ Perry A, Roth KA, Banerjee R, Fuller CE, Gutmann DH (July 2001). "NF1 deletions in S-100 protein-positive and negative cells of sporadic and neurofibromatosis 1 (NF1)-associated plexiform neurofibromas and malignant peripheral nerve sheath tumors". The American Journal of Pathology. 159 (1): 57–61. doi:10.1016/S0002-9440(10)61673-2. PMC 1850421. PMID 11438454.
- ^ Chaubal A, Paetau A, Zoltick P, Miettinen M (1994). "CD34 immunoreactivity in nervous system tumors". Acta Neuropathologica. 88 (5): 454–458. doi:10.1007/BF00389498. PMID 7531384.
- ^ Weiss SW, Langloss JM, Enzinger FM (September 1983). "Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors". Laboratory Investigation; A Journal of Technical Methods and Pathology. 49 (3): 299–308. PMID 6310227.
- ^ Carroll SL (March 2012). "Molecular mechanisms promoting the pathogenesis of Schwann cell neoplasms". Acta Neuropathologica. 123 (3): 321–348. doi:10.1007/s00401-011-0928-6. PMC 3288530. PMID 22160322.
- ^ Weiss SW, Nickoloff BJ (October 1993). "CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions". The American Journal of Surgical Pathology. 17 (10): 1039–1045. doi:10.1097/00000478-199310000-00009. PMID 7690524.