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vestibular_schwannoma

Vestibular schwannoma

A vestibular schwannoma (also known as acoustic neuroma, acoustic neurinoma, or acoustic neurilemoma) is a benign, usually slow-growing cerebellopontine angle tumor that develops from the balance and hearing nerves supplying the inner ear. The tumor comes from an overproduction of Schwann cells.

see also Spontaneous Vestibular Schwannoma Regression.

Epidemiology

Etiology

Classification

Scores

Clinical

Diagnosis

Differential diagnosis

Cerebellopontine angle meningioma.

Cerebellopontine hemangioblastoma.

Gao S et al., reported a cerebellar glioblastoma multiforme patient, with his clinical presentations and imaging characteristics mimicking a vestibular schwannoma. To the best of authors knowledge, this is the first reported patient with cGBM mimicking a vestibular schwannoma 1).

Guidelines

Treatment

Outcome

Case series

Case reports

A young man with cystic fibrosis in his early 30s presented to accident and emergency with acute onset unilateral lower motor neuron facial palsy, hearing loss and impaired balance following Mycobacterium abscessus eradication induction therapy. The hearing loss and impaired balance developed over a 3-day period prior to the onset of facial palsy. Further investigation with a CT scan and MRI scan led to a diagnosis of vestibular schwannoma. The facial palsy resolved with steroid treatment; however, the hearing loss is irreversible, which has had a profound impact on his life and career. This case is intriguing as the cause and association of events are unclear. A working diagnosis of incidental Bell's palsy and unilateral hearing loss caused by the vestibular schwannoma was applied. However, the onset of these symptoms in relation to M. abscessus eradication induction therapy promotes discussion 2).


Kritikos et al. encountered a case of DAVF associated with an octreotide-positive vestibular schwannoma. A 46-year-old female had symptoms of right ear congestion accompanied by pulsatile tinnitus and mild hearing loss. Magnetic resonance imaging (MRI) identified a lobulated mass centered at the cerebellopontine angle. Preoperatively, on cerebral angiography, there was an incidental discovery of a DAVF in the right posterior fossa. The decision was made to proceed with resection of the tumor in a staged fashion. Her latest follow-up MRI showed no evidence of recurrent tumor. This is the second reported case of DAVF associated with an intracranial schwannoma. Findings are discussed along with a thorough review of the literature. This case, combined with the data from the literature review, led us to believe that tumor-related angiogenesis might contribute to DAVF formation 3).

2015

Temporal bone invasion by VS is extremely rare. A 51-year-old man who revealed temporal bone destruction beyond IAC by unilateral VS. The bony destruction extended anteriorly to the carotid canal and inferiorly to the jugular foramen. On histopathologic examination, the tumor showed typical benign schwannoma and did not show any unusual vascularity or malignant feature. Facial nerve was severely compressed and distorted by tumor, which unevenly eroded temporal bone in surgical field. Vestibular schwannoma with atypical invasion of temporal bone can be successfully treated with combined translabyrinthine and lateral suboccipiral approach without facial nerve dysfunction. Early detection and careful dissection of facial nerve with intraoperative monitoring should be considered during operation due to severe adhesion and distortion of facial nerve by tumor and eroded temporal bone 4)


A 15-year-old male who presented with hearing loss due to a small left-sided vestibular schwannoma (VS) not associated with neurofibromatosis type 2 (NF2), which had been apparent for six months. Magnetic resonance imaging with gadolinium diethylenetriamine penta-acetic acid revealed a mass, 10 mm in diameter, located in the left inner auditory canal. The patient had no family history of NF2 and gene mutation analysis showed no signs of the condition. Small sporadic or non-NF2 VS is extremely rare and the treatment decision-making process is complicated in children when considering the implications for the impairment of childhood development and lifelong disability. Following careful consideration, the patient in the present study underwent treatment with stereotactic radiosurgery. The five-year post-operative follow-up examination showed tumor stability without additional neurological deficits and at the time of writing the patient was alive and well 5).

1)
Gao S, Liu X, Cheng P, Yuan X, Niu J, Bai Y, Xi B. A Primary Cerebellar Glioblastoma Multiforme Mimicking Vestibular Schwannoma. J Craniofac Surg. 2016 Aug 10. [Epub ahead of print] PubMed PMID: 27513787.
2)
Wilson C, Duckers J, Rajenderkumar D. Unusual discovery of a vestibular schwannoma following eradication therapy for Mycobacterium abscessus. BMJ Case Rep. 2018 Aug 29;2018. pii: bcr-2018-225572. doi: 10.1136/bcr-2018-225572. PubMed PMID: 30158267.
3)
Kritikos ME, Oselkin M, Sharma N, Gopal PP, Bigelow DC, Grady S, Hurst RW, Pukenas BA, Choudhri O, Kung D. Dural Arteriovenous Fistula Associated With a Vestibular Tumor: An Unusual Case and Review of the Literature. Cureus. 2018 Jun 27;10(6):e2890. doi: 10.7759/cureus.2890. PubMed PMID: 30159216; PubMed Central PMCID: PMC6110627.
4)
Park SJ, Yang NR, Seo EK. Vestibular schwannoma atypically invading temporal bone. J Korean Neurosurg Soc. 2015 Apr;57(4):292-4. doi: 10.3340/jkns.2015.57.4.292. Epub 2015 Apr 24. PubMed PMID: 25932298; PubMed Central PMCID: PMC4414775.
5)
Wang J, Xu Y, Lei T, Zeng L. Treatment decision-making for sporadic small vestibular schwannoma in a pediatric patient: A case report and literature review. Oncol Lett. 2015 May;9(5):2371-2373. Epub 2015 Mar 18. PubMed PMID: 26137073; PubMed Central PMCID: PMC4467327.
vestibular_schwannoma.txt · Last modified: 2018/09/04 13:51 by administrador