Intracranial schwannoma

Changed by Tim Luijkx, 13 Sep 2016

Updates to Article Attributes

Body was changed:

Intracranial schwannomas (also referred to as neurinomas) are common benign tumours, accounting for 6-8% of all intracranial tumours1

Schwannomas can occur essentially anywhere in the body. See also the general schwannoma article

Epidemiology

Schwannomas are most frequently encountered in middle aged and elderly adults with a predilection for females (F:M = 1.5-2:1), although they are found in all age groups. 

Clinical presentation

Clinical presentation depends on the both the size of the tumour, the nerve of origin and the exact location. In general they will present with symptoms pertaining to the parent cranial nerve. Larger tumours will also exert mass effect on adjacent structures. Specific features are discussed separately:

Multiple schwannomas, are a characteristic of neurofibromatosis type 2 (NF2). In this setting, any cranial nerve can be involved, including those that are very rarely seen in sporadic cases (e.g. oculomotor nerve) 2

Pathology

Two histological types of schwannomas are described 1,2:

  1. Antoni type A
    • cells are elongated and bipolar with unclear borders
    • arranged parallel to each other (palisade-like structures)
    • pure Antony type A tumours are typical of spinal schwannomas 
  2. Antoni type B
    • reticular structure
    • cells have lymphocyte like nuclei

Intracranial schwannomas typically have both Antony type A and B structure 1,2

Radiographic features

Although the appearance of individual schwannomas will be influenced by the nerve of origin, composition (Antoni type B typically demonstrate higher T2 signal) and surrounding anatomy, they naturally share similar imaging characteristics. 

Of note, the target sign which can be seen in neurogenic tumours elsewhere in the body (usually neurofibromas, but also sometimes in schwannomas) is essentially never encountered intracranially 2

CT

Schwannomas are typically isodense to brain, and can be difficult to identify, depending on location. Cystic areas (usually found in larger tumours) are hypodense, similar to CSF. Following administration of contrast they moderately enhance, often heterogeneously due to cystic areas 1,2.

Bone algorithm is excellent at assessing bony margins, especially useful when the tumour extends into or through a foramen. As these lesions are slow growing they remodel the bone, with smooth borders, rather than destroy the bone 1,2

MRI

MRI is the investigation of choice for assessment of intracranial schwannomas, not only due to greater contrast resolution, but also exquisite anatomical details, which allows for precise localisation of the tumour. This is particularly the case with high resolution T2 sequences (e.g. FIESTA, CISS, SPACE).

Typical signal characteristics are 1,2

  • T1
    • isointense to hypointense to brain
    • low signal cystic areas, if present
  • T1 C+ (Gd)
    • prominent enhancement
    • heterogenous in 70% of cases 1
  • T2
    • typically somewhat hyperintense to brain
    • cystic areas are hyperintense
  • T2* (GE / SWI)
    • hemosiderin staining may be enounctered, particularly in larger tumours 2
  • DWI/ADC
    • often higher signal on both DWI and ADC (T2 shine through - not restricted diffusion)

Treatment and prognosis

Although the details of therapeutic options will depend on the location of the tumour, size and patient circumstances, generally surgical resection is curative if complete resection is achieved. 

Radiotherapy is an alternative to surgery. 

Differential diagnosis

The differential diagnosis will depend mostly on the specific location of the tumour and which nerve it arises from. As such this is discussed separately in individual articles (see above). 

  • -<p><strong>Intracranial schwannomas</strong> (also referred to as <strong>neurinomas</strong>) are common benign tumours, accounting for 6-8% of all <a href="/articles/brain-tumours">intracranial tumours</a><sup>1</sup>. </p><p>Schwannomas can occur essentially anywhere in the body: see <a title="schwannoma" href="/articles/schwannoma">schwannoma</a>. </p><h4>Epidemiology</h4><p>Schwannomas are most frequently encountered in middle aged and elderly adults with a predilection for females (F:M = 1.5-2:1), although they are found in all age groups. </p><h4>Clinical presentation</h4><p>Clinical presentation depends on the both the size of the tumour, the nerve of origin and the exact location. In general they will present with symptoms pertaining to the parent cranial nerve. Larger tumours will also exert mass effect on adjacent structures. Specific features are discussed separately:</p><ul>
  • +<p><strong>Intracranial schwannomas</strong> (also referred to as <strong>neurinomas</strong>) are common benign tumours, accounting for 6-8% of all <a href="/articles/brain-tumours">intracranial tumours</a><sup>1</sup>. </p><p>Schwannomas can occur essentially anywhere in the body. See also the general <a href="/articles/schwannoma">schwannoma</a> article. </p><h4>Epidemiology</h4><p>Schwannomas are most frequently encountered in middle aged and elderly adults with a predilection for females (F:M = 1.5-2:1), although they are found in all age groups. </p><h4>Clinical presentation</h4><p>Clinical presentation depends on the both the size of the tumour, the nerve of origin and the exact location. In general they will present with symptoms pertaining to the parent cranial nerve. Larger tumours will also exert mass effect on adjacent structures. Specific features are discussed separately:</p><ul>
  • -</ul><p>Multiple schwannomas, are a characteristic of <a href="/articles/neurofibromatosis-type-2-3">neurofibromatosis type 2 (NF2)</a>. In this setting, any cranial nerve can be involved, including those that are very rarely seen in sporadic cases (e.g. <a title="Oculomotor nerve (CN III)" href="/articles/oculomotor-nerve">oculomotor nerve</a>) <sup>2</sup>. </p><h4>Pathology</h4><p>Two histological types of schwannomas are described <sup>1,2</sup>:</p><ol>
  • +</ul><p>Multiple schwannomas, are a characteristic of <a href="/articles/neurofibromatosis-type-2-3">neurofibromatosis type 2 (NF2)</a>. In this setting, any cranial nerve can be involved, including those that are very rarely seen in sporadic cases (e.g. <a href="/articles/oculomotor-nerve">oculomotor nerve</a>) <sup>2</sup>. </p><h4>Pathology</h4><p>Two histological types of schwannomas are described <sup>1,2</sup>:</p><ol>
  • -</ol><p>Intracranial schwannomas typically have both Antony type A and B structure <sup>1,2</sup>. </p><h4>Radiographic features</h4><p>Although the appearance of individual schwannomas will be influenced by the nerve of origin, composition (Antoni type B typically demonstrate higher T2 signal) and surrounding anatomy, they naturally share similar imaging characteristics. </p><p>Of note, the <a title="Target sign of neurofibromas" href="/articles/target-sign-of-neurofibromas">target sign</a> which can be seen in neurogenic tumours elsewhere in the body (usually <a title="neurofibroma" href="/articles/neurofibroma">neurofibromas</a>, but also sometimes in schwannomas) is essentially never encountered intracranially <sup>2</sup>. </p><h5>CT</h5><p>Schwannomas are typically isodense to brain, and can be difficult to identify, depending on location. Cystic areas (usually found in larger tumours) are hypodense, similar to CSF. Following administration of contrast they moderately enhance, often heterogeneously due to cystic areas <sup>1,2</sup>.</p><p>Bone algorithm is excellent at assessing bony margins, especially useful when the tumour extends into or through a foramen. As these lesions are slow growing they remodel the bone, with smooth borders, rather than destroy the bone <sup>1,2</sup>. </p><h5>MRI</h5><p>MRI is the investigation of choice for assessment of intracranial schwannomas, not only due to greater contrast resolution, but also exquisite anatomical details, which allows for precise localisation of the tumour. This is particularly the case with high resolution T2 sequences (e.g. FIESTA, CISS, SPACE).</p><p>Typical signal characteristics are <sup>1,2</sup>: </p><ul>
  • +</ol><p>Intracranial schwannomas typically have both Antony type A and B structure <sup>1,2</sup>. </p><h4>Radiographic features</h4><p>Although the appearance of individual schwannomas will be influenced by the nerve of origin, composition (Antoni type B typically demonstrate higher T2 signal) and surrounding anatomy, they naturally share similar imaging characteristics. </p><p>Of note, the <a href="/articles/target-sign-of-neurofibromas">target sign</a> which can be seen in neurogenic tumours elsewhere in the body (usually <a href="/articles/neurofibroma">neurofibromas</a>, but also sometimes in schwannomas) is essentially never encountered intracranially <sup>2</sup>. </p><h5>CT</h5><p>Schwannomas are typically isodense to brain, and can be difficult to identify, depending on location. Cystic areas (usually found in larger tumours) are hypodense, similar to CSF. Following administration of contrast they moderately enhance, often heterogeneously due to cystic areas <sup>1,2</sup>.</p><p>Bone algorithm is excellent at assessing bony margins, especially useful when the tumour extends into or through a foramen. As these lesions are slow growing they remodel the bone, with smooth borders, rather than destroy the bone <sup>1,2</sup>. </p><h5>MRI</h5><p>MRI is the investigation of choice for assessment of intracranial schwannomas, not only due to greater contrast resolution, but also exquisite anatomical details, which allows for precise localisation of the tumour. This is particularly the case with high resolution T2 sequences (e.g. FIESTA, CISS, SPACE).</p><p>Typical signal characteristics are <sup>1,2</sup>: </p><ul>

ADVERTISEMENT: Supporters see fewer/no ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.