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Meningioma

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Meningiomas are the most common extra-axial tumours of the central nervous system. They are a non-glial neoplasm that originates from the arachnoid cap cells of the meninges. Meningiomas have characteristic imaging findings although there are many variants. They are typically benign with a low recurrence rate but rarely can be malignant.

This article is a general discussion of meningiomas and focuses on the imaging findings of intracranial disease. For spinal disease refer to spinal meningioma.

Epidemiology

Meningiomas are more common in women, with a ratio of 2:1 intracranially and 4:1 in the spine. They are uncommon in patients before the age of 40 and should raise suspicion of neurofibromatosis type 2 (NF2) when found in young patients.

Clinical presentation

Many small meningiomas are found incidentally and are entirely asymptomatic. Often they cause concern as they are mistakenly deemed to be the cause of vague symptoms, most frequently headaches. Larger tumours or those with adjacent oedema or abutting particularly sensitive structures can present with a variety of symptoms. Most common presentations include ⁸:

headache: 36%
paresis: 22%
change in mental status: 21%
focal neurological deficits

Meningiomas may also become clinically apparent due to complications dependent on location including:

convexity/parasagittal: seizures and hemiparesis
basisphenoid: visual field defect
cavernous sinus: cranial nerve deficit(s)
frontal: anosmia (although often become very large before becoming symptomatic)
dural venous sinus invasion/dural venous sinus thrombosis (usually this occurs gradually and even occlusion is asymptomatic, with collateral veins having time to enlarge)
intraosseous extension: may be hyperostotic or osteolytic and may result in local mass effect (e.g. proptosis)

Pathology

Although the majority of tumours are sporadic, they are also seen in the setting of previous cranial irradiation and of course in patients withneurofibromatosis type II (NF2) (Merlin gene on Chromosome 22). Additionally meningiomas demonstrate oestrogen sensitivity and may grow during pregnancy.

They are also divided histologically into ^3,8:

meningothelial
fibroblastic: abundant reticulum and 'stout' collagen
transitional: whorl formation
syncytial: poorly formed polygonal cells arranged in lobules
angioblastic: now classified separately as a haemangiopericytoma
clear cell: high rate of local recurrence ⁶
psammomatous
microcystic ¹²
secretory
chordoid
lymphoplasmacyte-rich
metaplastic
papillary: has a high rate of local recurrence ⁸
rhabdoid: aggressive and have a very poor prognosis
mixed type

Macroscopic

In general, there are two main macroscopic forms:

globose: rounded, well defined dural masses, likened to the appearance of a fried egg seen in profile
en plaque: extensive regions of dural thickening

Microscopic

arise from meningothelial arachnoid cells
histological subtypes include:
- transitional
- fibroblastic
- syncytial
- psammomatous
- secretory
- microcytic
- papillary and rhabdoid: have a propensity to recur
haemangiopericytoma (CNS): previously angioblastic subtype

Variants

intraosseous meningioma: sclerotic or lucent
rarely degeneration into:
intraventricular meningioma
atypical meningioma (WHO II): have an increased mitotic rate, corresponds to ~ 7% of all meningiomas ⁴ and have a greater tendency to recur
malignant meningioma (WHO III)
- uncommon accounting for only 2.4% of all meningiomas ⁴ and demonstrate intraparenchymal invasion, rapid growth, and a high mitotic rate or sarcomatous degeneration
- like atypical meningiomas they too demonstrate restricted diffusion on DWI
- they are thought to originally be standard meningiomas which undergo malignant degeneration
- papillary subtype appears to do so more frequently than other
radiation induced meningioma
- more frequently multiple, and typically occur ~35 years after radiotherapy
- meningiomas are a much more frequent complication of radiotherapy compared to sarcomas or gliomas
clear cell meningioma: have up to a 60% recurrence rate and occur in younger patients ⁶
microcystic meningioma: rare, and are typically very high on T2 weighted imaging and are more commonly associated with atypical features and adjacent brain oedema ¹²

Grading

Generally follows the WHO classification for CNS tumours ^7,11:

WHO I: meningioma ~88-95 %
WHO II: atypical meningioma (atypical, clear cell, chordoid) ~ 5-6%
WHO III: malignant meningioma (rhabdoid, anaplastic, papillary) ~1%
WHO IV: meningioma with sarcomatous degeneration, extremely rare ¹¹

There is also a Simpson grade for meningiomas.

Radiographic features

Meningiomas are located anywhere that meninges are found, and in some places where only rest cells are presumed to be located. Locations include:

85-90% supratentorial ⁸
- 45% parasagittal, convexities
- 15-20% sphenoid ridge
- 10% olfactory groove/planum sphenoidale
- 5-10% juxtasellar
5-10% infratentorial
<5% miscellaneous intracranial
- intraventricular meningioma (choroid plexus)
- optic nerve meningioma
- pineal gland
- spinal: especially thoracic (see spinal meningioma)
<1% "extra dural"
- sinonasal cavity: most common
- intraosseous and may involve scalp
- parotid gland
- skin

Plain film

Plain films no longer have a role in the diagnosis or management of meningiomas. Historically a number of features were observed, including:

enlarged meningeal artery grooves
hyperostosis or lytic regions
calcification

CT

CT is often the first modality employed to investigate neurological signs or symptoms, and often is the modality which detects an incidental lesion:

60% slightly hyperdense to normal brain
20-30% have some calcification ⁸
72% brightly and homogeneously contrast enhance ⁸, less frequent in malignant or cystic variants
hyperostosis
- typical for meningiomas that abut the base of the skull
- need to distinguish reactive hyperostosis from skull vault invasion (eventually involves the outer table too)
lytic regions
pneumosinus dilatans

MRI

As is the case with most other intracranial pathology, MRI is the investigation of choice for the diagnosis and characterisation of meningiomas. When appearance and location is typical, the diagnosis can be made with a very high degree of certainty. In many instances however the appearances are atypical.

Meningiomas typically appear as extra-axial masses with a broad dural base. They are usually homogeneous and well circumscribed, although many variants are encountered.

Signal characteristics include:

T1
- isointense: ~60-90% ^{3,8, 13}
- somewhat hypointense: 10-40% compared to grey matter
T1 C+ (Gd): usually intense and homogenous enhancement
T2
- isointense: ~50% ^3,8,13
- hyperintense: 35-40%
  - usually correlates with soft textures and hypervascular tumours ¹³
  - very hyperintense lesions may represent the microcystic variant ¹²
- hypointense: 10-15% compared to grey matter
DWI: atypical and malignant subtypes may show greater than expected restricted diffusion although recent work suggests that this is not useful in prospectively predicting histological grade ^15-16

Helpful signs include:

CSF cleft sign, which is not specific for meningioma, but helps establish the mass to be extra-axial
dural tail seen in 60-72% ² (note that a dural tail is also seen in other processes)

Meningiomas typically narrow arteries which they encase. This is a useful sign to distinguish a meningioma from a pituitary macroadenoma which will not.

Oedema can be seen and correlates with:

size
rapid growth
location (convexity and parasagittal > elsewhere)
invasion in the case of malignant meningiomas

The underlying mechanisms for the oedema may relate to:

venous stasis/occlusion/thrombosis
compressive ischaemia
aggressive growth/invasion
parasitisation or pial vessles

MR spectroscopy (MRS)

Usually MRS does not play a significant role in diagnosis but can help distinguish meningiomas from mimics. Features include:

increase in alanine (1.3-1.5 ppm)
increased glutamine/glutamate
increased choline (Cho): cellular tumour
absent or significantly reduced N-acetylaspartate (NAA): non-neuronal origin
absent or significantly reduced creatine (Cr)

MR perfusion

good correlation between volume transfer constant (k-trans) and histological grade

Angiography

mother-in-law sign: "comes early, stays late, very dense", tumour blush
dual blood supply from both
- pial (ICA) supplies periphery
- meningeal vessels (ECA) supplies core
spoke wheel appearance
dense venous filling
preoperative embolisation: especially skull base, particles are favoured; 7-9 days prior to surgery

Treatment and prognosis

Treatment is usually with surgical excision. If only incomplete resection is possible (especially at the base of skull) then external-beam radiation therapy can be used ⁸.

Recurrence rate varies with grade and length of follow-up ⁸

WHO I: meningioma, 6.9%
WHO II: atypical meningioma, 34.6%
WHO III: malignant meningioma, 72.7%

5-year follow up: 5%
10-year follow up: 5%
32-year follow up: 5%

Metastatic disease is rare, but has been reported ⁸.

History and etymology

The term "meningioma" was first introduced by Harvey Cushing, neurosurgeon, in 1922 ⁹.

Differential diagnosis

The differential diagnosis largely depends on location:

cerebellopontine angle
- acoustic schwannoma
parasellar region
- pituitary macroadenoma
- craniopharyngioma
elsewhere

In the setting of hyperostosis consider:

In the setting of lucent intraossous meningioma the differential is essentially that of a solitary lucent lesion of the skull.

-<p><strong>Meningiomas</strong> are the most common extra-axial tumours of the <a href="/articles/central-nervous-system-curriculum">central nervous system</a>. They are a non-glial neoplasm that originates from the arachnoid cap cells of the <a href="/articles/meninges">meninges</a>. Meningiomas have characteristic imaging findings although there are many variants. They are typically benign with a low recurrence rate but rarely can be malignant. </p><p>This article is a general discussion of meningiomas and focuses on the imaging findings of intracranial disease. For spinal disease refer to <a href="/articles/spinal-meningioma">spinal meningioma</a>. </p><h4>Epidemiology</h4><p>Meningiomas are more common in women, with a ratio of 2:1 intracranially and 4:1 in the spine. They are uncommon in patients before the age of 40 and should raise suspicion of <a href="/articles/neurofibromatosis-type-2-3">neurofibromatosis type 2 (NF2)</a> when found in young patients.</p><h4>Clinical presentation</h4><p>Many small meningiomas are found incidentally and are entirely asymptomatic. Often they cause concern as they are mistakenly deemed to be the cause of vague symptoms, most frequently headaches. Larger tumours or those with adjacent oedema or abutting particularly sensitive structures can present with a variety of symptoms. Most common presentations include <sup>8</sup>:</p><ul>
+<p><strong>Meningiomas</strong> are the most common extra-axial tumours of the <a href="/articles/central-nervous-system-curriculum">central nervous system</a>. They are a non-glial neoplasm that originates from the arachnoid cap cells of the <a href="/articles/meninges">meninges</a>. Meningiomas have characteristic imaging findings although there are many variants. They are typically benign with a low recurrence rate but rarely can be malignant.</p><p>This article is a general discussion of meningiomas and focuses on the imaging findings of intracranial disease. For spinal disease refer to <a href="/articles/spinal-meningioma">spinal meningioma</a>.</p><h4>Epidemiology</h4><p>Meningiomas are more common in women, with a ratio of 2:1 intracranially and 4:1 in the spine. They are uncommon in patients before the age of 40 and should raise suspicion of <a href="/articles/neurofibromatosis-type-2-3">neurofibromatosis type 2 (NF2)</a> when found in young patients.</p><h4>Clinical presentation</h4><p>Many small meningiomas are found incidentally and are entirely asymptomatic. Often they cause concern as they are mistakenly deemed to be the cause of vague symptoms, most frequently headaches. Larger tumours or those with adjacent oedema or abutting particularly sensitive structures can present with a variety of symptoms. Most common presentations include <sup>8</sup>:</p><ul>
~~-</ul><p>Meningiomas may also become clinically apparent due to complications dependent on location including: </p><ul>~~
+</ul><p>Meningiomas may also become clinically apparent due to complications dependent on location including:</p><ul>
-</ul><h4>Pathology</h4><p>Although the majority of tumours are sporadic, they are also seen in the setting of previous cranial irradiation and of course in patients with<a href="/articles/nf2"> </a><a href="/articles/neurofibromatosis-type-2-3">neurofibromatosis type II (NF2)</a> (Merlin gene on Chromosome 22). Additionally meningiomas demonstrate oestrogen sensitivity and may grow during pregnancy. </p><p>They are also divided histologically into <sup>3,8</sup>:</p><ol>
+</ul><h4>Pathology</h4><p>Although the majority of tumours are sporadic, they are also seen in the setting of previous cranial irradiation and of course in patients with<a href="/articles/nf2"> </a><a href="/articles/neurofibromatosis-type-2-3">neurofibromatosis type II (NF2)</a> (Merlin gene on Chromosome 22). Additionally meningiomas demonstrate oestrogen sensitivity and may grow during pregnancy.</p><p>They are also divided histologically into <sup>3,8</sup>:</p><ol>
~~-<li>rhabdoid: aggressive and have a very poor prognosis </li>~~
+<li>rhabdoid: aggressive and have a very poor prognosis</li>
~~-<li>~~
~~-<a href="/articles/psammomatous-meningioma">psammomatous</a> </li>~~
+<li><a href="/articles/psammomatous-meningioma">psammomatous</a></li>
~~-<a href="/articles/malignant-meningioma">malignant meningioma</a> (WHO III) <ul>~~
+<a href="/articles/malignant-meningioma">malignant meningioma</a> (WHO III) <ul>
~~-<li>WHO II: <a href="/articles/atypical-meningioma">atypical meningioma</a> (atypical, clear cell, chordoid) ~ 5-6% </li>~~
+<li>WHO II: <a href="/articles/atypical-meningioma">atypical meningioma</a> (atypical, clear cell, chordoid) ~ 5-6%</li>
~~-<li>WHO IV: meningioma with sarcomatous degeneration, extremely rare <sup>11</sup> </li>~~
-</ul><p>There is also a <a href="/articles/simpson-grade">Simpson grade</a> for meningiomas.</p><h4>Radiographic features</h4><p>Meningiomas are located anywhere that meninges are found, and in some places where only rest cells are presumed to be located. Locations include: </p><ul>
+<li>WHO IV: meningioma with sarcomatous degeneration, extremely rare <sup>11</sup>
+</li>
+</ul><p>There is also a <a href="/articles/simpson-grade">Simpson grade</a> for meningiomas.</p><h4>Radiographic features</h4><p>Meningiomas are located anywhere that meninges are found, and in some places where only rest cells are presumed to be located. Locations include:</p><ul>
~~-</ul><h5>Plain film </h5><p>Plain films no longer have a role in the diagnosis or management of meningiomas. Historically a number of features were observed, including:</p><ul>~~
+</ul><h5>Plain film</h5><p>Plain films no longer have a role in the diagnosis or management of meningiomas. Historically a number of features were observed, including:</p><ul>
~~-<li><a title="pneumosinus dilatans" href="/articles/pneumosinus-dilatans">pneumosinus dilatans</a></li>~~
-</ul><h5>MRI</h5><p>As is the case with most other intracranial pathology, MRI is the investigation of choice for the diagnosis and characterisation of meningiomas. When appearance and location is typical, the diagnosis can be made with a very high degree of certainty. In many instances however the appearances are atypical. </p><p>Meningiomas typically appear as extra-axial masses with a broad dural base. They are usually homogeneous and well circumscribed, although many variants are encountered. </p><p>Signal characteristics include:</p><ul>
+<li><a href="/articles/pneumosinus-dilatans">pneumosinus dilatans</a></li>
+</ul><h5>MRI</h5><p>As is the case with most other intracranial pathology, MRI is the investigation of choice for the diagnosis and characterisation of meningiomas. When appearance and location is typical, the diagnosis can be made with a very high degree of certainty. In many instances however the appearances are atypical.</p><p>Meningiomas typically appear as extra-axial masses with a broad dural base. They are usually homogeneous and well circumscribed, although many variants are encountered.</p><p>Signal characteristics include:</p><ul>

Case 3315: planum sphenoidale meningioma

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meningioma","thumbnail":"https://prod-images-static.radiopaedia.org/images/63478092/faa4373a0afe83b1746f881bdb77f6d6d942549f5009f1bb447623fb2953bb28_big_gallery.jpeg","isStack":true,"diagnosticCertainty":"confirmed_unsubstantiated"},{"imageId":64787486,"studyId":146489,"caseSlug":"meningioma-120","caption":"Case 33","thumbnail":"https://prod-images-static.radiopaedia.org/images/64787486/9b3c24072a2568610b99707aa719ce850ac5b3ac515d6abb3692649109cf19e9_big_gallery.jpeg","isStack":true,"diagnosticCertainty":"confirmed_unsubstantiated"}],"ddx_inclusions":[{"imageId":54663308,"studyId":104378,"caseSlug":"prostate-metastasis-to-sphenoid-bone-and-orbit","caption":"Prostate metastasis to sphenoid bone and orbit","thumbnail":"https://prod-images-static.radiopaedia.org/images/54663308/5._big_gallery.jpeg","isStack":true,"diagnosticCertainty":"confirmed_unsubstantiated"}],"lang":"us"}

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