Vestibular tumor
Template:Vestibular tumor
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Aditya Ganti M.B.B.S. [2]
Overview
Vestibular tumors are growths that tend to develop underneath the serous tissue of the sublinguinal region. They may be found anywhere between the chin and the larynx (or voicebox) and are not more inclined to one side of the body than the other. They are predominantly present in adolescent females though they are not directly related to any hygienal issues. While surgery is the most often cure, deaths rarely occur due to the existence of vestibular tumors.
Historical Perspective
The first reported case of a vestibular tumor was in 1898 in Lancaster, Pennsylvania. Though there have been stories of growths of the like of vestibular tumors, this was the first medically reported case. At the time, surgery was too dangerous, so Emilia Walfen was forced to live with the tumor, which eventually grew to the size of a Concord grape.
Pathogenesis
Recent studies in NF2 patients led to the identification of the neurofibromin 2 gene, which is located on chromosome 22. The NF2 gene produces merlin, also known as schwannomin, a cell membrane-related protein that acts as a tumor suppressor. Biallelic inactivation of the NF2 gene is found in most sporadic vestibular schwannomas.
Microscopic pathology
- Vestibular schwannomas arise from perineural elements of the Schwann cell.
- They occur with equal frequency on the superior and inferior branches of the vestibular nerve.
- Microscopically, zones of alternately dense and sparse cellularity, called Antoni A and B areas, respectively, are characteristic of vestibular schwannomas.
- Malignant degeneration is extremely rare, with only six cases having been reported.
- Immunohistochemical staining for S100 protein is usually positive in both the benign and the rare malignant forms of this tumor.
Differentiating Vestibular schwannoma from other diseases
The differential diagnosis includes meningioma, facial nerve schwannomas, gliomas, cholesterol cysts, cholesteatomas, hemangiomas, aneurysms, arachnoid cysts, lipomas, and metastatic tumor. For more information click here On the basis of seizure, visual disturbance, and constitutional symptoms, meningioma must be differentiated from oligodendroglioma, astrocytoma, hemangioblastoma, pituitary adenoma, schwannoma, 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 | ||||||||||
Meningioma [1][2][3] |
+ | +/− | +/− | − | + | − |
|
|
| |
Glioblastoma multiforme [4][5][6] |
+ | +/− | +/− | − | + | − |
|
|
| |
Oligodendroglioma [7][8][9] |
+ | + | +/− | − | + | − |
|
|
| |
Hemangioblastoma [10][11][12][13] |
+ | +/− | +/− | − | + | − |
|
| ||
Pituitary adenoma [14][15][6] |
− | − | + Bitemporal hemianopia | − | − |
|
|
|
| |
Schwannoma [16][17][18][19] |
− | − | − | − | + | − |
|
|
| |
Primary CNS lymphoma [20][21] |
+ | +/− | +/− | − | + | − |
|
|
| |
Childhood primary brain tumors | ||||||||||
Pilocytic astrocytoma [22][23][24] |
+ | +/− | +/− | − | + | − |
|
|
| |
Medulloblastoma [25][26][27] |
+ | +/− | +/− | − | + | − |
|
|
| |
Ependymoma [28][6] |
+ | +/− | +/− | − | + | − |
|
|
| |
Craniopharyngioma [29][30][31][6] |
+ | +/− | + Bitemporal hemianopia | − | + |
|
|
|
| |
Pinealoma [32][33][34] |
+ | +/− | +/− | − | + vertical gaze palsy |
|
|
|
| |
Vascular | ||||||||||
AV malformation [35][36][6] |
+ | + | +/− | − | +/− | − |
|
| ||
Brain aneurysm [37][38][39][40][41] |
+ | +/− | +/− | − | +/− | − |
|
|
|
|
Infectious | ||||||||||
Bacterial brain abscess [42][43] |
+ | +/− | +/− | + | + |
|
|
|
|
|
Tuberculosis [44][6][45] |
+ | +/− | +/− | + | + |
|
|
|
|
|
Toxoplasmosis [46][47] |
+ | +/− | +/− | − | + |
|
|
|
|
|
Hydatid cyst [48][6] |
+ | +/− | +/− | +/− | + |
|
|
|
|
|
CNS cryptococcosis [49] |
+ | +/− | +/− | + | + |
|
|
|
|
|
CNS aspergillosis [50] |
+ | +/− | +/− | + | + |
|
|
|
|
|
Other | ||||||||||
Brain metastasis [51][6] |
+ | +/− | +/− | + | + | − |
|
|
|
|
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
Risk Factors
Common risk factors for the development of vestibular schwaomas include:
- Childhood exposure to low-dose radiation for benign conditions of the head and neck
- Radiofrequency radiation from the use of mobile phones
- Noise exposure
Epidemiology and Demographics
Incidence
- The overall incidence of vestibular schwannomas is approximately 1 per 100,000 person-years in the United States.
- Bilateral vestibular schwannomas are primarily observed in patients with neurofibromatosis type 2 (NF2).
- The tumors are unilateral in more than 90 percent of cases, affecting the right and left sides with equal frequency.
Age
- The median age at diagnosis is approximately 50 years.
Gender
- Vestibular schwannomas occur equally in both genders.
Natural History, Complications, and Prognosis
Vestibular schwanama pose a major health impediment if left untreated as they might cause pressure on adjacent posterior fossa structures such as cerebellum or brainstem and result in ataxia Brainstem compression, cerebellar tonsil herniation, hydrocephalus, and death can occur in untreated cases. Common complication include seizures and paralysis difficulty swallowing due to the pressure on the tongue or pharynx. The functions of the lower cranial nerves can also become impaired, leading to dysarthria, dysphagia, aspiration, and hoarseness.
Diagnosis
History and Symptoms
- Symptoms associated with vestibular schwannoma can be due to cranial nerve involvement, cerebellar compression, or tumor progression. Clinical manifestations in this series included the following:
Never involvement | Incidence | Symtpoms |
---|---|---|
Cochlear nerve | 95 percent |
|
Vestibular nerve | 61 percent |
|
Trigeminal nerve | 17 percent |
|
Facial nerve | 6 percent |
|
Tumor progression |
|
Physical Examination
- Hearing tests are typically abnormal due to involvement of the acoustic nerve.
- The Weber and Rinne tests may be useful in suggesting asymmetric sensorineural hearing impairment.
- Neurologic examination may reveal other cranial nerve deficits
- A decreased or absent ipsilateral corneal reflex and facial twitching or hypesthesia may occur as cranial nerves V and VII become affected.
- Romberg, Hall-Pike, and other common office balance tests are typically normal.
CT
Findings of vestibular schwanoma on CT include:
- Erosion and widening of the internal acoustic canal.
- The density of these tumors on non-contrast imaging is variable, and often they are hard to see, especially on account of beam hardening and streak artefact from the adjacent petrous temporal bone.
- Contrast enhancement is present but can be underwhelming, especially in larger lesions with cystic components.
MRI
MRI findings of vestibular schwanoma include:
MRI findings of Vestibular Schwanoma | |
---|---|
T1 |
|
T2 |
|
T1 C+ (Gd) | Contrast enhancement is intense however, heterogeneous in larger tumors |
Other Diagnostic Studies
Audiometry
- Audiometry is the best initial screening laboratory test for the diagnosis of vestibular schwannoma.
- Pure tone and speech audiometry should be performed in an acoustically shielded area.
- Test results typically show an asymmetric sensorineural hearing loss, usually more prominent in the higher frequencies.
- Hearing loss does not necessarily correlate with tumor size.
- The speech discrimination score is usually markedly reduced in the affected ear and out of proportion to the measured hearing loss.
- Common audiometry tests that are of current practice include:
- Acoustic reflex testing,
- Impedance audiometry
- Bekesy audiometry.
- Brainstem-evoked response audiometry (AER/ABR).
Vestibular testing
- Vestibular testing has limited utility as a screening test for the diagnosis of vestibular schwannoma because of the accuracy of evoked response audiometry.
- When testing is performed, a decreased or absent caloric response on the affected side may be seen. When the tumor is small, though, a normal response is often seen.
Treatment
Treatment options for patients with a vestibular schwannoma include surgery and radiation therapy.
Surgery
Surgery generally results in satisfactory long-term control of vestibular schwannomas. There are three standard operative approaches.
Surgery | |
---|---|
Retromastoid suboccipital (retrosigmoid) | The suboccipital approach can be used for any size tumor with or without attempted hearing preservation. |
Translabyrinthine | The translabyrinthine approach has been recommended for acoustic tumors larger than 3 cm and for smaller tumors when hearing preservation is not an issue. |
Middle fossa | The middle fossa approach is suitable for small (<1.5 cm) tumors when hearing preservation is a goal. |
Radiation therapy
Radiation therapy for patients with vestibular schwannoma include stereotactic radiosurgery (SRS), stereotactic radiotherapy (SRT), and proton beam therapy, as well as conventional fractionated radiation therapy.
Radiation therapy | |
---|---|
Stereotactic radiosurgery |
|
Stereotactic radiotherapy |
|
Proton beam therapy |
|
References
- ↑ 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.
- ↑ 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.
- ↑ 6.0 6.1 6.2 6.3 6.4 6.5 6.6 6.7 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.