Loading
A vestibular schwannoma is a benign brain tumour, a growth on the balance and hearing nerve, deep in the skull. It is rarely dangerous, so many are simply watched with scans; growing tumours are treated with either an operation or a single precisely focused dose of radiation, and people with the inherited condition NF2, who develop tumours on both sides, can be helped by the drug bevacizumab.
Vestibular schwannoma is a WHO grade 1 nerve sheath tumour arising from Schwann cells of the vestibular division of the eighth cranial nerve in the internal auditory canal and cerebellopontine angle. Sporadic tumours are unilateral and carry somatic NF2 inactivation; bilateral tumours define NF2-related schwannomatosis, the germline condition renamed in 2022 from neurofibromatosis type 2, which also causes meningiomas, spinal schwannomas and ependymomas. Presentation is progressive unilateral hearing loss, tinnitus and imbalance; large tumours compress the brainstem and cause hydrocephalus. Diagnosis is by MRI; biopsy is not needed.
Management has three arms. Observation with serial MRI is appropriate for small tumours because many do not grow, and hearing can be followed. Microsurgery through retrosigmoid, translabyrinthine or middle fossa approaches removes the tumour with facial nerve monitoring; it is favoured for large tumours with brainstem compression and in younger patients. Stereotactic radiosurgery at marginal doses of about 12 to 13 Gy controls the great majority of small and medium tumours with low facial nerve risk, and fractionated stereotactic radiotherapy or proton therapy is used for larger lesions. Leksell treated the first acoustic neuroma with the Gamma Knife in 1969, and radiosurgery has since displaced surgery for most small growing tumours. Hearing preservation is the outcome that most separates the options and depends on pre-treatment hearing and tumour size.
In NF2-related schwannomatosis the aim is to preserve hearing and function over a lifetime of multiple tumours. Bevacizumab produced tumour shrinkage and hearing improvement in progressive NF2 tumours (Plotkin and colleagues, NEJM 2009) and is used off label; brigatinib showed activity across NF2-related tumour types in the INTUITT-NF2 platform trial (2024), and cochlear or auditory brainstem implants restore hearing after bilateral loss. Radiosurgery is used more cautiously in NF2 because control rates are lower and there is a small concern about malignant change in a germline-predisposed nerve.
A benign tumour found in roughly one in a thousand people over a lifetime, presenting with one-sided hearing loss; sporadic tumours are single, while NF2-related schwannomatosis causes bilateral tumours from young adulthood and is the hard form of the disease.
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Papillary tumour of the pineal region, Choroid plexus carcinoma, Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma, Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Observation with MRI and audiometry, typically yearly; treatment on documented growth or hearing decline.
Stereotactic radiosurgery (Gamma Knife, CyberKnife or linac) at about 12 to 13 Gy, or fractionated stereotactic radiotherapy; microsurgery in younger patients or by preference.
Microsurgical resection with facial nerve monitoring, sometimes deliberately subtotal followed by radiosurgery to the remnant; shunting for hydrocephalus.
Bevacizumab (off label, phase 2 evidence) to shrink tumours and improve hearing; brigatinib on INTUITT-NF2 evidence; hearing rehabilitation with cochlear or auditory brainstem implants; surgery and radiosurgery used selectively.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Brigatinib is the first drug shown to act across the tumour types of NF2-related schwannomatosis and is now offered for progressive tumours; the platform design lets further drugs be tested against the same benchmark.
The observation-first approach and the framing of treatment as a trade between facial nerve function, hearing and tumour control on the vestibular schwannoma page reflect this review.
Bevacizumab is used off label for NF2-related schwannomatosis with growing tumours or declining hearing, supported by later phase 2 trials, and the study opened the search for medical therapy of schwannoma.
Query for this cancer: (TITLE:"Vestibular schwannoma" OR ABSTRACT:"Vestibular schwannoma" OR TITLE:"acoustic neuroma" OR ABSTRACT:"acoustic neuroma" OR TITLE:"Acoustic neuroma" OR ABSTRACT:"Acoustic neuroma" OR TITLE:"NF2-related schwannomatosis" OR ABSTRACT:"NF2-related schwannomatosis" OR TITLE:"Eighth nerve tumour" OR ABSTRACT:"Eighth nerve tumour") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Vestibular schwannoma (acoustic neuroma), not a curated reading list.
Among the first successful removals of a cerebellopontine angle tumour.
The operating microscope transforms mortality and facial nerve outcomes.
Trofatter and Rouleau identify the merlin tumour suppressor on chromosome 22.
Plotkin and colleagues (NEJM).
Plotkin and colleagues (NEJM) report the first platform trial in the condition.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
The shared side effects of drugs that block blood vessel growth (bevacizumab, ramucirumab and VEGFR kinase inhibitors): high blood pressure, protein leaking into the urine, nosebleeds and more serious bleeding, slow wound healing, and rarely holes in the bowel.
Death of brain tissue months to years after radiosurgery or high-dose brain radiotherapy, which can look exactly like tumour growing back on a scan.
Blood clots in the leg veins or lungs. Cancer makes blood clot more easily and some treatments (IMiDs, anti-VEGF drugs, hormone therapy, central lines, surgery) add risk; clots are the second commonest cause of death in cancer patients after the cancer itself.
See all on the product pages:BevacizumabBrigatinib·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Vestibular schwannoma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.