Acoustic schwannomas (a.k.a. vestibular schwannomas) are relatively common tumours that arise from the vestibulocochlear nerve (CN VIII) and represent ~80% of cerebellopontine angle masses. Bilateral acoustic schwannomas are strongly suggestive of neurofibromatosis type 2 (NF2).
These tumours classically present on imaging as a solid nodular mass with an intracanalicular component that often result in widening of the porus acusticus. They usually have vivid contrast enhancement and, when larger, cystic degeneration can be present. Haemorrhagic areas may also be seen, but calcification is typically not present.
They account for 7-8% of all primary intracranial tumours 2 and 75-90% of cerebellopontine angle masses 1-2,8. Bilateral vestibular schwannomas are highly suggestive of neurofibromatosis type 2 (NF2), although bilateral tumours are encountered in the familial form of acoustic schwannomas in the absence of other stigmata of NF2 5.
The typical presentation is with adult-onset sensorineural hearing loss or tinnitus. In some patients this goes unnoticed and presentation is delayed until the lesion is much larger and presents with mass effect. Possibilities include cerebellar and brainstem symptoms (e.g. other cranial nerve dysfunction), or hydrocephalus due to effacement of the fourth ventricle.
Acoustic schwannomas are benign tumours (WHO grade 1), which usually arise from the intracanalicular segment of the vestibular portion of the vestibulocochlear nerve (CN VIII) 2,4, near the transition point between glial and Schwann cells (Obersteiner-Redlich zone) 8. In over 90% of cases these tumours arise from the inferior division of the vestibular nerve 8.
They are well circumscribed encapsulated masses which, unlike neuromas, arise from but are separate from nerve fibres 7, which they usually spray and displace rather than incorporate.
They can display two types of growth pattern:
- elongated cells with cytoplasmic processes arranged in fascicles 7
- little stromal matrix
- Verocay bodies: nuclear-free zones of processes lying between regions of nuclear palisading
- loose meshwork of cells
- less densely cellular
- microcysts and myxoid change
Most vestibular schwannomas have an intracanalicular component, and often result in widening of the porus acusticus resulting in the trumpeted IAM sign, which is present in up to 90% of cases 5. In a minority of cases (~20%) they are purely extra canalicular, only abutting the porus acousticus 1,5. Usually there is a small CSF cap between the intracanalicular portion and the cochlea; Occasionally, these tumours grow laterally through the cochlea (transmodiolar) or vestibule (transmacular) into the middle ear. Rarely they are small and confined to the vestibule (intravestibular) or the cochlea (intracochlear) or both (vestibulocochlear) 4.
Extracanalicular extension into the cerebellopontine angle (path of least resistance) can lead to "ice-cream-cone" appearance.
Small tumours tend to be solid whereas cystic degeneration seen commonly in larger tumours 2. Haemorrhagic areas may also be seen. Calcification is typically not present.
May show erosion and widening of the internal acoustic canal. The density of these tumours on non-contrast imaging is variable, and often they are hard to see, especially on account of beam hardening and streak artefact form the adjacent petrous temporal bone.
Contrast enhancement is present, but can be underwhelming, especially in larger lesions with cystic components.
- slightly hypointense cf. adjacent brain (63%) 2
- isointense cf. adjacent brain (37%) 2
- may contain hypointense cystic areas
- heterogeneously hyperintense cf. to adjacent brain 5
- cystic areas fluid intensity
- may have associated peritumoural arachnoid cysts 3
T1 C+ (Gd)
- contrast enhancement is vivid
- but heterogeneous in larger tumours
Linear enhancement may not indicate tumour, but if there is nodular enhancement suspect tumour recurrence (needs follow up MRI).
Treatment and prognosis
There is variability in the rate of growth of these tumours, and as such the decision to treat requires consideration of patient's age and co-morbidities. The options include 6 :
- observation and follow-up
- stereotactic radiosurgery
- microsurgery: number of approaches are possible, including 8:
- retrosigmoid (trans-meatal) (suboccipital)
- able to preserve hearing
- can be used for large tumours
- limited view of the IAC
- has a greater chance of residual tumour (in the lateral aspect of IAC)
middle cranial fossa
- best for small intracanalicular tumours
- able to preserve hearing
- careful skeletonisation of the facial nerve required
- loss of hearing guaranteed
- retrosigmoid (trans-meatal) (suboccipital)
Overall tumour recurrence is low, ranging between 1 and 9% 8.
The most frequent differential to be considered are:
- usually more homogeneous in appearance: significant signal heterogeneity with cystic or haemorrhagic areas is more typical of vestibular schwannoma than meningiomas (although cystic meningiomas do occur)
- meningiomas tend to have a broad dural base
- usually lack trumpet IAM sign
- calcification more common
- no enhancing component
- very high signal on DWI
- does not widen the IAC
- usually does not remodel the IAC as metastases are usually present for only a short time
- centered on the fourth ventricle
- does not extend into the IAC
- usually younger patients
What the surgeon wants to know
In addition to general remarks about the size and location of the tumour, significant findings that influence surgical management include 8:
- how close to the fundus of the IAC the tumour reaches
- anatomical variations
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