Schwannoma
For patient information click here
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Synonyms and keywords: Neurilemmoma, Neurinoma, Neurolemmoma, Schwann cell tumor, Schwannomatosis
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]
Overview
Schwannoma may be classified according to pathology into 4 subtypes: conventional schwannoma, cellular schwannoma, plexiform schwannoma, and melanotic schwannoma. Based on location it is classified into intracranial schwannoma,Acoustic neuroma (most common),trigeminal schwannoma, facial nerve schwannoma. Schwannomas are composed of spindle cells which demonstrate two growth patterns Antoni type A and Antoni type B. Antoni type A patternin which elongated cells are densely packed and arranged in fascicles. Palisades are sometimes seen, when prominent these form verocay bodies. Antoni type B pattern cells are less compact and are prone to cystic degeneration. Schwannoma variants include ancient schwannoma, cellular schwannoma, melanotic schwannoma, plexiform schwannoma. The incidence vestibular schwannoma is approximately 1 per 100,000 individuals. People of all age groups may develop vestibular schwannoma. The incidence is more common between the 3rd and 5th decades of life. The median age at diagnosis is 5th decade.Symptoms of intracranial schwannoma include hearing loss, tinnitus, dysphagia, ataxia, Vertigo, Facial weakness, Dizziness, spinal schwannomas present with Back pain, Urinary incontinence, Urinary retention, Clumsiness, Weakness, Paresthesias. Surgery is the mainstay of treatment for schwannoma. There are three main approaches like translybyrinthine, retrosigmoid, middle fossa. The common complications of surgery include vertigo, hearing loss is another important complication associated with the operation, post-operative headache, cerebrospinal fluid (CSF) leakage, facial paralysis
Historical perspective
Classification
Schwannoma may be classified according to pathology into 4 subtypes: conventional schwannoma, cellular schwannoma, plexiform schwannoma, and melanotic schwannoma. Based on location it is classified into intracranial schwannoma,Acoustic neuroma (most common),trigeminal schwannoma, facial nerve schwannoma, jugular foramen schwannoma, hypoglossal schwannomas, spinal schwannoma, intercostal nerve schwannoma, intramuscular schwannoma, posterior mediastinum schwannoma, retroperitoneum schwannoma, intracerebral schwannoma.
Pathophysiology
Schwannomas may arise sporadically or in association with Neurofibromatosis type 2 as a result of mutations involving merlin protein. Loss of function of a tumor suppressor gene called merlin gene is noted commonly. Schwannomas are composed of spindle cells which demonstrate two growth patterns Antoni type A and Antoni type B. Antoni type A patternin which elongated cells are densely packed and arranged in fascicles. Palisades are sometimes seen, when prominent these form verocay bodies. Antoni type B pattern cells are less compact and are prone to cystic degeneration. Schwannoma variants include ancient schwannoma, cellular schwannoma, melanotic schwannoma, plexiform schwannoma. Immunohistochemistry positive for S100, collagen IV, CD34, neurofilament protein, podoplanin, calretinin, Sox10. Associated conditions include neurofibromatosis type 2, schwannomatosis, carney’s complex.
Causes
Epidemiology and Demographics
The incidence vestibular schwannoma is approximately 1 per 100,000 individuals. People of all age groups may develop vestibular schwannoma. The incidence is more common between the 3rd and 5th decades of life. The median age at diagnosis is 5th decade. The incidence seems to be higher in Asian population and lower in Hispanics and African-Americans. Vestibular schwannoma affects men and women equally with a slight predilection towards female population.
Risk Factors
Screening
Differentiating Schwannoma from other Diseases
On the basis of seizure, visual disturbance, and constitutional symptoms, schwannoma must be differentiated from oligodendroglioma, meningioma, hemangioblastoma, pituitary adenoma, astrocytoma, primary CNS lymphoma, medulloblastoma, ependymoma, craniopharyngioma, pinealoma, AV malformation, brain aneurysm, bacterial brain abscess, tuberculosis, toxoplasmosis, hydatid cyst, CNS cryptococcosis, CNS aspergillosis, and brain metastasis.
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Symptoms of intracranial schwannoma include hearing loss, tinnitus, dysphagia, ataxia, Vertigo, Facial weakness, Dizziness, spinal schwannomas present with Back pain, Urinary incontinence, Urinary retention, Clumsiness, Weakness, Paresthesias
Physical Examination
X-Ray
There are no X-ray findings associated with schwannoma.
CT
CT findings of schwannoma include low to intermediate attenuation, intense contrast enhancement, small tumors typically demonstrate homogeneous enhancement and larger tumors may show heterogeneous enhancement, adjacent bone remodelling with smooth corticated edges
MRI
Schwannomas appear on T1 as isointense or hypointense, T1 C+ (Gd) intense enhancement,T2- heterogeneously hyperintense (Antoni A: relatively low, Antoni B: high), cystic degenerative areas may be present, especially in larger tumors, T2- larger tumors often have areas of hemosiderin. Signs can also be useful in diagnosing such as split-fat sign: thin peripheral rim of fat best seen on planes along long axis of the lesion in non-fat-suppressed sequences, target sign: peripheral high T2 signal, central low signal rarely seen intracranially, fascicular sign: multiple small ring-like structures.
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
The feasibility of surgery depends on the stage of schwannoma at diagnosis. Surgery is the mainstay of treatment for schwannoma. There are three main approaches like translybyrinthine, retrosigmoid, middle fossa. The common complications of surgery include vertigo, hearing loss is another important complication associated with the operation, post-operative headache, cerebrospinal fluid (CSF) leakage, facial paralysis
Primary Prevention
There are no primary preventive methods available for schwannoma.
Secondary Prevention
References
Historical Perspective
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]
Overview
Historical Perspective
Discovery
- There is limited information about the historical perspective of [disease name].
OR
- [Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].
- The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
- In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
- In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].
Landmark Events in the Development of Treatment Strategies
Impact on Cultural History
Famous Cases
The following are a few famous cases of [disease name]:
References
Classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
classification
Schwannoma may be classified according to pathology into 4 subtypes: conventional schwannoma, cellular schwannoma, plexiform schwannoma, and melanotic schwannoma.
| Classification | |
|---|---|
| Pathology |
|
| Location |
|
References
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: ; Maneesha Nandimandalam, M.B.B.S.[2]
Overview
Schwannomas may arise sporadically or in association with Neurofibromatosis type 2 as a result of mutations involving merlin protein. Loss of function of a tumor suppressor gene called merlin gene is noted commonly. Schwannomas are composed of spindle cells which demonstrate two growth patterns Antoni type A and Antoni type B. Antoni type A pattern in which elongated cells are densely packed and arranged in fascicles. Palisades are sometimes seen, when prominent these form verocay bodies. Antoni type B pattern cells are less compact and are prone to cystic degeneration. Schwannoma variants include ancient schwannoma, cellular schwannoma, melanotic schwannoma, plexiform schwannoma. Immunohistochemistry positive for S100, collagen IV, CD34, neurofilament protein, podoplanin, calretinin, Sox10. Associated conditions include neurofibromatosis type 2, schwannomatosis, carney’s complex.
Pathophysiology
Pathogenesis
- Unilateral schwannomas are usually sporadic. [1]
- Bilateral schwannomas are associated with Neurofibromatosis type 2.
- Neoplastic proliferation of schwann cell differentiation leading to tumor cells growing diffusely within and along the nerves affecting the neural elements. [2]
- Those associated with Neurofibromatosis type 2 are due to deletion of NF2 locus (22q12.2) which encodes a tumor suppressor protein, merlin (schwannomin).
- Loss of merlin expression affects the cell cycle and mitogenic signal pathways.
Genetics
- Loss of function of a tumor suppressor gene called merlin gene, either by:
- Direct genetic change involving the NF2 gene on chromosome 22 [3]
- Secondarily to merlin inactivation
Associated Conditions
- Neurofibromatosis type 2 (NF2)[4]
- Schwannomatosis
- Carney’s complex
Gross and Microscopic Pathology
Microscopic appearance
- Conventional schwannomas are composed of spindle cells which demonstrate two growth patterns: Antoni type A and Antoni type B.[5][6][7]
- Antoni type A pattern: elongated cells are densely packed and arranged in fascicles. Palisades are sometimes seen; when prominent these form Verocay bodies.
- Antoni type B pattern cells are less compact and are prone to cystic degeneration.
Variants
Schwannoma variants include:
- ancient schwannoma
- cellular schwannoma
- predominantly composed of Antoni A tissue
- no Verocay bodies
- most commonly found in a paravertebral location, or trigeminal nerves (CN V)
- melanotic schwannoma: dense melanin pigment
- plexiform schwannoma[8]
- usually arise from skin or subcutaneous tissues
- usually diagnosed at birth or childhood
- usually sporadic, but rarely associated with NF2
- should not be confused with plexiform neurofibromas
- associated with NF1
- may undergo malignant change
Immunohistochemistry
- S100
- Collagen IV
- CD34
- Neurofilament protein
- Podoplanin
- Calretinin
- Sox10
Negative for:
- EMA
References
- ↑ Hadfield KD, Smith MJ, Urquhart JE, Wallace AJ, Bowers NL, King AT, Rutherford SA, Trump D, Newman WG, Evans DG (November 2010). “Rates of loss of heterozygosity and mitotic recombination in NF2 schwannomas, sporadic vestibular schwannomas and schwannomatosis schwannomas”. Oncogene. 29 (47): 6216–21. doi:10.1038/onc.2010.363. PMID 20729918.
- ↑ Fisher ER, Vuzevski VD (February 1968). “Cytogenesis of schwannoma (neurilemoma), neurofibroma, dermatofibroma, and dermatofibrosarcoma as revealed by electron microscopy”. Am. J. Clin. Pathol. 49 (2): 141–54. doi:10.1093/ajcp/49.2.141. PMID 5639539.
- ↑ Hadfield KD, Smith MJ, Urquhart JE, Wallace AJ, Bowers NL, King AT, Rutherford SA, Trump D, Newman WG, Evans DG (November 2010). “Rates of loss of heterozygosity and mitotic recombination in NF2 schwannomas, sporadic vestibular schwannomas and schwannomatosis schwannomas”. Oncogene. 29 (47): 6216–21. doi:10.1038/onc.2010.363. PMID 20729918.
- ↑ Hilton DA, Hanemann CO (April 2014). “Schwannomas and their pathogenesis”. Brain Pathol. 24 (3): 205–20. doi:10.1111/bpa.12125. PMID 24450866.
- ↑ Doddrell RD, Dun XP, Shivane A, Feltri ML, Wrabetz L, Wegner M; et al. (2013). “Loss of SOX10 function contributes to the phenotype of human Merlin-null schwannoma cells”. Brain. 136 (Pt 2): 549–63. doi:10.1093/brain/aws353. PMC 3572932. PMID 23413263.
- ↑ Sayed SI, Rane P, Deshmukh A, Chaukar D, Menon S, Arya S; et al. (2012). “Ancient schwannoma of the parapharynx causing dysphagia: a rare entity”. Ann R Coll Surg Engl. 94 (7): e217–20. doi:10.1308/003588412X13373405385737. PMC 3954264. PMID 23031754.
- ↑ Giovannini M, Bonne NX, Vitte J, Chareyre F, Tanaka K, Adams R; et al. (2014). “mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma”. Neuro Oncol. 16 (4): 493–504. doi:10.1093/neuonc/not242. PMC 3956353. PMID 24414536.
- ↑ Tchernev G, Chokoeva AA, Patterson JW, Bakardzhiev I, Wollina U, Tana C (February 2016). “Plexiform Neurofibroma: A Case Report”. Medicine (Baltimore). 95 (6): e2663. doi:10.1097/MD.0000000000002663. PMC 4753888. PMID 26871793.
- ↑ Rodriguez FJ, Folpe AL, Giannini C, Perry A (March 2012). “Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems”. Acta Neuropathol. 123 (3): 295–319. doi:10.1007/s00401-012-0954-z. PMC 3629555. PMID 22327363.
- ↑ Shintaku M (September 2011). “Immunohistochemical localization of autophagosomal membrane-associated protein LC3 in granular cell tumor and schwannoma”. Virchows Arch. 459 (3): 315–9. doi:10.1007/s00428-011-1104-z. PMC 3162629. PMID 21674156.
Causes
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [3]; Associate Editor(s)-in-Chief: ; Maneesha Nandimandalam, M.B.B.S.[4]
Overview
The cause of schwannoma has not been identified.
Causes
Life-threatening Causes
- There are no life-threatening causes for schwannoma.
Common Causes
- Mutations involving merlin protein.
Genetic Causes
- Loss of function of a tumor suppressor gene called merlin gene
References
References
Differentiating Schwannoma from other Diseases
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D.
Overview
On the basis of seizure, visual disturbance, and constitutional symptoms, schwannoma must be differentiated from oligodendroglioma, meningioma, hemangioblastoma, pituitary adenoma, astrocytoma, primary CNS lymphoma, medulloblastoma, ependymoma, craniopharyngioma, pinealoma, AV malformation, brain aneurysm, bacterial brain abscess, tuberculosis, toxoplasmosis, hydatid cyst, CNS cryptococcosis, CNS aspergillosis, and brain metastasis.
Differentiating schwannoma from other Diseases
Differentiating schwannoma from other diseases on the basis of seizure, visual disturbance, and constitutional symptoms
On the basis of seizure, visual disturbance, and constitutional symptoms, schwannoma must be differentiated from oligodendroglioma, meningioma, hemangioblastoma, pituitary adenoma, astrocytoma, primary CNS lymphoma, medulloblastoma, ependymoma, craniopharyngioma, pinealoma, AV malformation, brain aneurysm, bacterial brain abscess, tuberculosis, toxoplasmosis, hydatid cyst, CNS cryptococcosis, CNS aspergillosis, and brain metastasis.
| Diseases | Clinical manifestations | Para-clinical findings | Gold standard |
Additional findings | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Symptoms | Physical examination | |||||||||
| Lab Findings | MRI | Immunohistopathology | ||||||||
| Head- ache |
Seizure | Visual disturbance | Constitutional | Focal neurological deficit | ||||||
| Adult primary brain tumors | ||||||||||
| Schwannoma [1][2][3][4] |
− | − | − | − | + | − |
|
|
| |
| Glioblastoma multiforme [5][6][7] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Oligodendroglioma [8][9][10] |
+ | + | +/− | − | + | − |
|
|
| |
| Meningioma [11][12][13] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Hemangioblastoma [14][15][16][17] |
+ | +/− | +/− | − | + | − |
|
| ||
| Pituitary adenoma [18][19][7] |
− | − | + Bitemporal hemianopia | − | − |
|
|
|
| |
| Primary CNS lymphoma [20][21] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Childhood primary brain tumors | ||||||||||
| Pilocytic astrocytoma [22][23][24] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Medulloblastoma [25][26][27] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Ependymoma [28][7] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Craniopharyngioma [29][30][31][7] |
+ | +/− | + Bitemporal hemianopia | − | + |
|
|
|
| |
| Pinealoma [32][33][34] |
+ | +/− | +/− | − | + vertical gaze palsy |
|
|
|
| |
| Vascular | ||||||||||
| AV malformation [35][36][7] |
+ | + | +/− | − | +/− | − |
|
| ||
| Brain aneurysm [37][38][39][40][41] |
+ | +/− | +/− | − | +/− | − |
|
|
|
|
| Infectious | ||||||||||
| Bacterial brain abscess [42][43] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| Tuberculosis [44][7][45] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| Toxoplasmosis [46][47] |
+ | +/− | +/− | − | + |
|
|
|
|
|
| Hydatid cyst [48][7] |
+ | +/− | +/− | +/− | + |
|
|
|
|
|
| CNS cryptococcosis [49] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| CNS aspergillosis [50] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| Other | ||||||||||
| Brain metastasis [51][7] |
+ | +/− | +/− | + | + | − |
|
|
|
|
ABBREVIATIONS
CNS=Central nervous system, AV=Arteriovenous, CSF=Cerebrospinal fluid, NF-2=Neurofibromatosis type 2, MEN-1=Multiple endocrine neoplasia, GFAP=Glial fibrillary acidic protein, HIV=Human immunodeficiency virus, BhCG=Human chorionic gonadotropin, ESR=Erythrocyte sedimentation rate, AFB=Acid fast bacilli, MRA=Magnetic resonance angiography, CTA=CT angiography
References
- ↑ Donnelly, Martin J.; Daly, Carmel A.; Briggs, Robert J. S. (2007). “MR imaging features of an intracochlear acoustic schwannoma”. The Journal of Laryngology & Otology. 108 (12). doi:10.1017/S0022215100129056. ISSN 0022-2151.
- ↑ Feany MB, Anthony DC, Fletcher CD (May 1998). “Nerve sheath tumours with hybrid features of neurofibroma and schwannoma: a conceptual challenge”. Histopathology. 32 (5): 405–10. PMID 9639114.
- ↑ Chen H, Xue L, Wang H, Wang Z, Wu H (July 2017). “Differential NF2 Gene Status in Sporadic Vestibular Schwannomas and its Prognostic Impact on Tumour Growth Patterns”. Sci Rep. 7 (1): 5470. doi:10.1038/s41598-017-05769-0. PMID 28710469.
- ↑ Hardell, Lennart; Hansson Mild, Kjell; Sandström, Monica; Carlberg, Michael; Hallquist, Arne; Påhlson, Anneli (2003). “Vestibular Schwannoma, Tinnitus and Cellular Telephones”. Neuroepidemiology. 22 (2): 124–129. doi:10.1159/000068745. ISSN 0251-5350.
- ↑ Sathornsumetee S, Rich JN, Reardon DA (November 2007). “Diagnosis and treatment of high-grade astrocytoma”. Neurol Clin. 25 (4): 1111–39, x. doi:10.1016/j.ncl.2007.07.004. PMID 17964028.
- ↑ Pedersen CL, Romner B (January 2013). “Current treatment of low grade astrocytoma: a review”. Clin Neurol Neurosurg. 115 (1): 1–8. doi:10.1016/j.clineuro.2012.07.002. PMID 22819718.
- ↑ 7.0 7.1 7.2 7.3 7.4 7.5 7.6 7.7 Mattle, Heinrich (2017). Fundamentals of neurology : an illustrated guide. Stuttgart New York: Thieme. ISBN 9783131364524.
- ↑ Smits M (2016). “Imaging of oligodendroglioma”. Br J Radiol. 89 (1060): 20150857. doi:10.1259/bjr.20150857. PMC 4846213. PMID 26849038.
- ↑ Wesseling P, van den Bent M, Perry A (June 2015). “Oligodendroglioma: pathology, molecular mechanisms and markers”. Acta Neuropathol. 129 (6): 809–27. doi:10.1007/s00401-015-1424-1. PMC 4436696. PMID 25943885.
- ↑ Kerkhof M, Benit C, Duran-Pena A, Vecht CJ (2015). “Seizures in oligodendroglial tumors”. CNS Oncol. 4 (5): 347–56. doi:10.2217/cns.15.29. PMC 6082346. PMID 26478444.
- ↑ Zee CS, Chin T, Segall HD, Destian S, Ahmadi J (June 1992). “Magnetic resonance imaging of meningiomas”. Semin. Ultrasound CT MR. 13 (3): 154–69. PMID 1642904.
- ↑ Shibuya M (2015). “Pathology and molecular genetics of meningioma: recent advances”. Neurol. Med. Chir. (Tokyo). 55 (1): 14–27. doi:10.2176/nmc.ra.2014-0233. PMID 25744347.
- ↑ Begnami MD, Palau M, Rushing EJ, Santi M, Quezado M (September 2007). “Evaluation of NF2 gene deletion in sporadic schwannomas, meningiomas, and ependymomas by chromogenic in situ hybridization”. Hum. Pathol. 38 (9): 1345–50. doi:10.1016/j.humpath.2007.01.027. PMC 2094208. PMID 17509660.
- ↑ Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH (May 2014). “Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease”. J. Neurosurg. 120 (5): 1055–62. doi:10.3171/2014.1.JNS131431. PMC 4762041. PMID 24579662.
- ↑ Hussein MR (October 2007). “Central nervous system capillary haemangioblastoma: the pathologist’s viewpoint”. Int J Exp Pathol. 88 (5): 311–24. doi:10.1111/j.1365-2613.2007.00535.x. PMC 2517334. PMID 17877533.
- ↑ Lee SR, Sanches J, Mark AS, Dillon WP, Norman D, Newton TH (May 1989). “Posterior fossa hemangioblastomas: MR imaging”. Radiology. 171 (2): 463–8. doi:10.1148/radiology.171.2.2704812. PMID 2704812.
- ↑ Perks WH, Cross JN, Sivapragasam S, Johnson P (March 1976). “Supratentorial haemangioblastoma with polycythaemia”. J. Neurol. Neurosurg. Psychiatry. 39 (3): 218–20. PMID 945331.
- ↑ Kucharczyk W, Davis DO, Kelly WM, Sze G, Norman D, Newton TH (December 1986). “Pituitary adenomas: high-resolution MR imaging at 1.5 T”. Radiology. 161 (3): 761–5. doi:10.1148/radiology.161.3.3786729. PMID 3786729.
- ↑ Syro LV, Scheithauer BW, Kovacs K, Toledo RA, Londoño FJ, Ortiz LD, Rotondo F, Horvath E, Uribe H (2012). “Pituitary tumors in patients with MEN1 syndrome”. Clinics (Sao Paulo). 67 Suppl 1: 43–8. PMC 3328811. PMID 22584705.
- ↑ Chinn RJ, Wilkinson ID, Hall-Craggs MA, Paley MN, Miller RF, Kendall BE, Newman SP, Harrison MJ (December 1995). “Toxoplasmosis and primary central nervous system lymphoma in HIV infection: diagnosis with MR spectroscopy”. Radiology. 197 (3): 649–54. doi:10.1148/radiology.197.3.7480733. PMID 7480733.
- ↑ Paulus, Werner (1999). “Classification, Pathogenesis and Molecular Pathology of Primary CNS Lymphomas”. Journal of Neuro-Oncology. 43 (3): 203–208. doi:10.1023/A:1006242116122. ISSN 0167-594X.
- ↑ Sathornsumetee S, Rich JN, Reardon DA (November 2007). “Diagnosis and treatment of high-grade astrocytoma”. Neurol Clin. 25 (4): 1111–39, x. doi:10.1016/j.ncl.2007.07.004. PMID 17964028.
- ↑ Pedersen CL, Romner B (January 2013). “Current treatment of low grade astrocytoma: a review”. Clin Neurol Neurosurg. 115 (1): 1–8. doi:10.1016/j.clineuro.2012.07.002. PMID 22819718.
- ↑ Mattle, Heinrich (2017). Fundamentals of neurology : an illustrated guide. Stuttgart New York: Thieme. ISBN 9783131364524.
- ↑ Dorwart, R H; Wara, W M; Norman, D; Levin, V A (1981). “Complete myelographic evaluation of spinal metastases from medulloblastoma”. Radiology. 139 (2): 403–408. doi:10.1148/radiology.139.2.7220886. ISSN 0033-8419.
- ↑ Fruehwald-Pallamar, Julia; Puchner, Stefan B.; Rossi, Andrea; Garre, Maria L.; Cama, Armando; Koelblinger, Claus; Osborn, Anne G.; Thurnher, Majda M. (2011). “Magnetic resonance imaging spectrum of medulloblastoma”. Neuroradiology. 53 (6): 387–396. doi:10.1007/s00234-010-0829-8. ISSN 0028-3940.
- ↑ Burger, P. C.; Grahmann, F. C.; Bliestle, A.; Kleihues, P. (1987). “Differentiation in the medulloblastoma”. Acta Neuropathologica. 73 (2): 115–123. doi:10.1007/BF00693776. ISSN 0001-6322.
- ↑ Yuh, E. L.; Barkovich, A. J.; Gupta, N. (2009). “Imaging of ependymomas: MRI and CT”. Child’s Nervous System. 25 (10): 1203–1213. doi:10.1007/s00381-009-0878-7. ISSN 0256-7040.
- ↑ Brunel H, Raybaud C, Peretti-Viton P, Lena G, Girard N, Paz-Paredes A, Levrier O, Farnarier P, Manera L, Choux M (September 2002). “[Craniopharyngioma in children: MRI study of 43 cases]”. Neurochirurgie (in French). 48 (4): 309–18. PMID 12407316.
- ↑ Prabhu, Vikram C.; Brown, Henry G. (2005). “The pathogenesis of craniopharyngiomas”. Child’s Nervous System. 21 (8–9): 622–627. doi:10.1007/s00381-005-1190-9. ISSN 0256-7040.
- ↑ Kennedy HB, Smith RJ (December 1975). “Eye signs in craniopharyngioma”. Br J Ophthalmol. 59 (12): 689–95. PMC 1017436. PMID 766825.
- ↑ Ahmed SR, Shalet SM, Price DA, Pearson D (September 1983). “Human chorionic gonadotrophin secreting pineal germinoma and precocious puberty”. Arch. Dis. Child. 58 (9): 743–5. PMID 6625640.
- ↑ Sano, Keiji (1976). “Pinealoma in Children”. Pediatric Neurosurgery. 2 (1): 67–72. doi:10.1159/000119602. ISSN 1016-2291.
- ↑ Baggenstoss, Archie H. (1939). “PINEALOMAS”. Archives of Neurology And Psychiatry. 41 (6): 1187. doi:10.1001/archneurpsyc.1939.02270180115011. ISSN 0096-6754.
- ↑ Kucharczyk, W; Lemme-Pleghos, L; Uske, A; Brant-Zawadzki, M; Dooms, G; Norman, D (1985). “Intracranial vascular malformations: MR and CT imaging”. Radiology. 156 (2): 383–389. doi:10.1148/radiology.156.2.4011900. ISSN 0033-8419.
- ↑ Fleetwood, Ian G; Steinberg, Gary K (2002). “Arteriovenous malformations”. The Lancet. 359 (9309): 863–873. doi:10.1016/S0140-6736(02)07946-1. ISSN 0140-6736.
- ↑ Chapman, Arlene B.; Rubinstein, David; Hughes, Richard; Stears, John C.; Earnest, Michael P.; Johnson, Ann M.; Gabow, Patricia A.; Kaehny, William D. (1992). “Intracranial Aneurysms in Autosomal Dominant Polycystic Kidney Disease”. New England Journal of Medicine. 327 (13): 916–920. doi:10.1056/NEJM199209243271303. ISSN 0028-4793.
- ↑ Castori M, Voermans NC (October 2014). “Neurological manifestations of Ehlers-Danlos syndrome(s): A review”. Iran J Neurol. 13 (4): 190–208. PMC 4300794. PMID 25632331.
- ↑ Schievink, W. I.; Raissi, S. S.; Maya, M. M.; Velebir, A. (2010). “Screening for intracranial aneurysms in patients with bicuspid aortic valve”. Neurology. 74 (18): 1430–1433. doi:10.1212/WNL.0b013e3181dc1acf. ISSN 0028-3878.
- ↑ Germain DP (May 2017). “Pseudoxanthoma elasticum”. Orphanet J Rare Dis. 12 (1): 85. doi:10.1186/s13023-017-0639-8. PMC 5424392. PMID 28486967.
- ↑ Farahmand M, Farahangiz S, Yadollahi M (October 2013). “Diagnostic Accuracy of Magnetic Resonance Angiography for Detection of Intracranial Aneurysms in Patients with Acute Subarachnoid Hemorrhage; A Comparison to Digital Subtraction Angiography”. Bull Emerg Trauma. 1 (4): 147–51. PMC 4789449. PMID 27162847.
- ↑ Haimes, AB; Zimmerman, RD; Morgello, S; Weingarten, K; Becker, RD; Jennis, R; Deck, MD (1989). “MR imaging of brain abscesses”. American Journal of Roentgenology. 152 (5): 1073–1085. doi:10.2214/ajr.152.5.1073. ISSN 0361-803X.
- ↑ Brouwer, Matthijs C.; Tunkel, Allan R.; McKhann, Guy M.; van de Beek, Diederik (2014). “Brain Abscess”. New England Journal of Medicine. 371 (5): 447–456. doi:10.1056/NEJMra1301635. ISSN 0028-4793.
- ↑ Morgado, Carlos; Ruivo, Nuno (2005). “Imaging meningo-encephalic tuberculosis”. European Journal of Radiology. 55 (2): 188–192. doi:10.1016/j.ejrad.2005.04.017. ISSN 0720-048X.
- ↑ Be NA, Kim KS, Bishai WR, Jain SK (March 2009). “Pathogenesis of central nervous system tuberculosis”. Curr. Mol. Med. 9 (2): 94–9. PMC 4486069. PMID 19275620.
- ↑ Chinn RJ, Wilkinson ID, Hall-Craggs MA, Paley MN, Miller RF, Kendall BE, Newman SP, Harrison MJ (December 1995). “Toxoplasmosis and primary central nervous system lymphoma in HIV infection: diagnosis with MR spectroscopy”. Radiology. 197 (3): 649–54. doi:10.1148/radiology.197.3.7480733. PMID 7480733.
- ↑ Helton KJ, Maron G, Mamcarz E, Leventaki V, Patay Z, Sadighi Z (November 2016). “Unusual magnetic resonance imaging presentation of post-BMT cerebral toxoplasmosis masquerading as meningoencephalitis and ventriculitis”. Bone Marrow Transplant. 51 (11): 1533–1536. doi:10.1038/bmt.2016.168. PMID 27348541.
- ↑ Taslakian B, Darwish H (September 2016). “Intracranial hydatid cyst: imaging findings of a rare disease”. BMJ Case Rep. 2016. doi:10.1136/bcr-2016-216570. PMC 5030532. PMID 27620198.
- ↑ McCarthy M, Rosengart A, Schuetz AN, Kontoyiannis DP, Walsh TJ (July 2014). “Mold infections of the central nervous system”. N. Engl. J. Med. 371 (2): 150–60. doi:10.1056/NEJMra1216008. PMC 4840461. PMID 25006721.
- ↑ McCarthy M, Rosengart A, Schuetz AN, Kontoyiannis DP, Walsh TJ (July 2014). “Mold infections of the central nervous system”. N. Engl. J. Med. 371 (2): 150–60. doi:10.1056/NEJMra1216008. PMC 4840461. PMID 25006721.
- ↑ Pope WB (2018). “Brain metastases: neuroimaging”. Handb Clin Neurol. 149: 89–112. doi:10.1016/B978-0-12-811161-1.00007-4. PMC 6118134. PMID 29307364.
Epidemiology and Demographics
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]
Overview
The incidence vestibular schwannoma is approximately 1 per 100,000 individuals. People of all age groups may develop vestibular schwannoma. The incidence is more common between the 3rd and 5th decades of life. The median age at diagnosis is 5th decade. The incidence seems to be higher in Asian population and lower in Hispanics and African-Americans. Vestibular schwannoma affects men and women equally with a slight predilection towards female population.
Epidemiology and demographics
Incidence
- The incidence vestibular schwannoma is approximately 1 per 100,000 individuals.
Age
- Patients of all age groups may develop vestibular schwannoma.
- The incidence is more common between the 3rd and 5th decades of life.
- The median age at diagnosis is 5th decade.[1]
Race
- The incidence seems to be higher in Asian population and lower in Hispanics and African-Americans.[2]
Gender
- Vestibular schwannoma affects men and women equally with a slight predilection towards female population. [3]
References
- ↑ Propp JM, McCarthy BJ, Davis FG, Preston-Martin S (January 2006). “Descriptive epidemiology of vestibular schwannomas”. Neuro-oncology. 8 (1): 1–11. doi:10.1215/S1522851704001097. PMC 1871924. PMID 16443943.
- ↑ Koo M, Lai JT, Yang EY, Liu TC, Hwang JH (October 2018). “Incidence of Vestibular Schwannoma in Taiwan from 2001 to 2012: A Population-Based National Health Insurance Study”. Ann. Otol. Rhinol. Laryngol. 127 (10): 694–697. doi:10.1177/0003489418788385. PMID 30032646.
- ↑ Brown CM, Ahmad ZK, Ryan AF, Doherty JK (January 2011). “Estrogen receptor expression in sporadic vestibular schwannomas”. Otol. Neurotol. 32 (1): 158–62. doi:10.1097/MAO.0b013e3181feb92a. PMC 3073320. PMID 21099731.
Risk Factors
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: ; Maneesha Nandimandalam, M.B.B.S.[2]
Overview
Common risk factors in the development of schwannoma are related to occupational, environmental, and genetic factors such as exposure to loud noise or cranial imaging, history of head injury or epilepsy or alcohol use.
Risk Factors
Common Risk Factors
- Common risk factors in the development of schwannoma include:
Less Common Risk Factors
- Less common risk factors in the development of schwannoma include:
- Head injury
- Alcohol
- Cancer
References
- ↑ Chen M, Fan Z, Zheng X, Cao F, Wang L (May 2016). “Risk Factors of Acoustic Neuroma: Systematic Review and Meta-Analysis”. Yonsei Med. J. 57 (3): 776–83. doi:10.3349/ymj.2016.57.3.776. PMC 4800371. PMID 26996581.
- ↑ Corona AP, Ferrite S, Lopes Mda S, Rêgo MA (April 2012). “Risk factors associated with vestibular nerve schwannomas”. Otol. Neurotol. 33 (3): 459–65. doi:10.1097/MAO.0b013e3182487fee. PMID 22377646.
- ↑ Berkowitz O, Iyer AK, Kano H, Talbott EO, Lunsford LD (December 2015). “Epidemiology and Environmental Risk Factors Associated with Vestibular Schwannoma”. World Neurosurg. 84 (6): 1674–80. doi:10.1016/j.wneu.2015.07.007. PMID 26171891.
Screening
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]
Overview
There is insufficient evidence to recommend routine screening for schwannoma.
Screening
There is insufficient evidence to recommend routine screening for schwannoma.
References
Natural History, Complications and Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Maneesha Nandimandalam, M.B.B.S.[2]
Natural history
More than half of all VS grow at an average of 2–4 mm/year, whereas less than 10% regress27. One study revealed that extrameatal tumors (28.9%) were more likely to grow compared to intrameatal tumors (17%) and a larger percentage of tumors grew early on after detection28. VS >2 cm are more likely to grow compared to smaller VS29–30. Growth rates of >2 mm/year are associated with decreased rates of hearing preservation compared to slower growth rates31
Clinical presentation
Presentation depends on the location of the tumor (see below) but generally, symptoms are due to local mass effect or dysfunction of the nerve they arise from. [1]
Symptoms of schwannoma depend on the location of the tumor.
- Intracranial schwannoma:
- Acoustic neuroma (most common)
- Sensorineural hearing loss
- Vertigo
- Tinnitus
- Facial weakness
- Facial numbness and tingling
- Headaches
- Dizziness
- Difficulty swallowing and hoarseness
- Taste changes
- Confusion
- Trigeminal schwannoma
- Trigeminal nerve dysfunction
- Facial nerve schwannoma
- Facial nerve dysfunction
- Jugular foramen schwannoma
- Hypoglossal schwannomas
- Hypoglossal nerve dysfunction
- Spinal schwannoma
- Intercostal nerve schwannoma
- Usually asymptomatic
- Intramuscular schwannoma
- Painless mass
Complications
Besides the complications that are directly produced the schwannoma itself due to it mass affect, there are many other complications that may arise post-surgical or post-radiation therapy depending on the anatomic location of the tumor. [2]
Surgical complications:
- Peri-operative hemorrhage
- CSF leaks
- Infections
Prognosis
- Schwannomas are slow growing tumors.
- The rate of recurrence after resection is extremely low. [3]
- They almost never turn malignant.
References
- ↑ Matthies C, Samii M (January 1997). “Management of 1000 vestibular schwannomas (acoustic neuromas): clinical presentation”. Neurosurgery. 40 (1): 1–9, discussion 9–10. doi:10.1097/00006123-199701000-00001. PMID 8971818.
- ↑ Yashar P, Zada G, Harris B, Giannotta SL (September 2012). “Extent of resection and early postoperative outcomes following removal of cystic vestibular schwannomas: surgical experience over a decade and review of the literature”. Neurosurg Focus. 33 (3): E13. doi:10.3171/2012.7.FOCUS12206. PMID 22937847.
- ↑ Senapati SB, Mishra SS, Dhir MK, Patnaik A, Panigrahi S (2016). “Recurrence of spinal schwannoma: Is it preventable?”. Asian J Neurosurg. 11 (4): 451. doi:10.4103/1793-5482.145060. PMC 4974985. PMID 27695564.
Diagnosis
Diagnosis
Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | [Schwannoma interventions|Interventions]] | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
Related Chapters
Related Chapters
- Neurofibroma
- Vestibular schwannoma (Acoustic neuroma)
Looking for the patient version?
© 2026 MyEClinic – IFTM Institut für Telematik in der Medizin GmbH