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Meningioma  Edit article Share article

Dr Bahman Rasuli ◉ and Assoc Prof Frank Gaillard ◉ ◈ et al.

Article information
Meningiomas are extra-axial tumours and represent the most common tumour of the meninges.
They are a non-glial neoplasm that originates from the meningocytes or arachnoid cap cells of the ADVERTISEMENT: Supporters see fewer/no ads
meninges and are located anywhere that meninges are found, and in some places where only rest
cells are presumed to be located. Although they are usually easily diagnosed and are typically
benign with a low rate of recurrence following surgery, there are a large number of histological
variants with variable imaging features and, in some instances, more aggressive biological Cases and gures
behaviour.

A broad division of meningiomas is into primary intradural (which may or may not have a secondary
extradural extension) and primary extradural (rare) 18 . They can also be classi ed according to the
location (e.g. spinal, intraosseous, intraventricular, etc.), by histological variants (e.g. clear cell,
rhabdoid, etc.), and by aetiology (e.g. radiation-induced, etc.).

Typical meningiomas appear as dural-based masses isointense to grey matter on both T1 and T2
weighted imaging enhancing vividly on both MRI and CT. Some of the variants as mentioned earlier
can, however, vary dramatically in their imaging appearance. Case 1: falcine
Figure 1: gross pathology
meningioma
This article is a general discussion of meningioma focussing on typical primary intradural
meningiomas and the imaging ndings of intracranial disease. For spinal and primary extradural
tumours refer to spinal meningioma and primary extradural meningioma articles respectively. Many
of the histological variants are also discussed separately. 

Rarely (e.g. 1-2% of cases 27 ) meningiomas may also arise at ectopic sites (ectopic primary
meningioma) such as in head and neck, orbit, nose, paranasal sinus, oropharynx and even places
such as the lung.

Epidemiology Case 2: olfactory

groove meningioma
Case 3: frontal
meningioma

Meningiomas are more common in women, with a ratio of 2:1 intracranially and 4:1 in the spine.
Atypical and malignant meningiomas are slightly more common in males. They are uncommon in
patients before the age of 40 and should raise suspicion of neuro bromatosis type 2 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
Case 5: intraventricular
headaches. Larger tumours or those with adjacent oedema or abutting particularly sensitive Case 4
meningioma
structures can present with a variety of symptoms. Most common presentations include 8 :

headache: 36%
paresis: 22%
change in mental status: 21%

Meningiomas may also become clinically apparent due to mass e ect depending on their location:

supratentorial: 85-90% 8
parasagittal, convexities: 45% Case 7:
Case 6
seizures and hemiparesis cerebellopontine ang…

sphenoid ridge: 15-20%
olfactory groove/planum sphenoidale: 10%
anosmia (usually not recognised)
Foster Kennedy syndrome
juxtasellar: 5-10%
visual eld defects
cranial nerve de cits
infratentorial: 5-10% Case 8 Case 9: dural tail

obstructive hydrocephalus
cranial nerve de cits
miscellaneous intradural: <5%
intraventricular meningioma
optic nerve meningioma
pineal gland
Parinaud syndrome
obstructive hydrocephalus Case 11: multiple
Case 10
meningiomas and schwa…
Occasionally transosseous or intraosseous involvement with prominent hyperostosis may result in
local mass e ect (e.g. proptosis).

Although dural venous sinus invasion and occlusion does occur, it usually occurs very gradually.
Therefore most cases of venous invasion are asymptomatic as collateral veins have had time to
enlarge.

Pathology
Meningiomas are thought to arise from meningocytes or arachnoid cap cells, which themselves Case 13: petrous ridge
Case 12
arise from pluripotent mesenchymal progenitor cells, which accounts for the unusual location of meningioma
primary extradural tumours 18,19 .

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

Grading
Grading of meningiomas follows the WHO classi cation for CNS tumours and includes both usual
Case 14: posterior fossa Case 15: suprasellar
histological features (e.g. mitotic index) as well as a number of histological subtypes, some of which meningioma meningioma
have been associated with more aggressive behaviour 7,11,23 :

grade I: 'benign" (70%)
transitional meningioma (40%): mixed histology, typically containing meningothelial and
brous components
meningothelial meningioma (17%)
brous meningioma (7%)
microcystic meningioma
psammomatous meningioma Case 16 Case 17
angiomatous meningioma *
secretory meningioma
metaplastic meningioma
lymphoplasmacytic-rich meningioma
grade II: "atypical" (30%)
clear cell meningioma
chordoid meningioma
atypical by histological criteria (29%) 11
Case 18: clivus Case 19: chordoid
4 to 19 mitoses per ten high-power elds meningioma meningioma

in ltration into brain parenchyma **

3 or more of the following 5 histologic features: necrosis, sheet-like growth, small cell
change, increased cellularity, prominent nucleoli
grade III: "anaplastic" or "malignant" (~1%)
rhabdoid meningioma
papillary meningioma
anaplastic by histological criteria 11
obvious malignant features similar to those seen in melanoma, carcinoma, or high-
Case 20 Case 21
grade sarcoma
20 or more mitosis per ten high-power elds

* Haemangiopericytomas were, until 1993, considered angiomatous meningiomas, but in 2007 WHO
classi cation of CNS tumours they were classi ed as a separate entity under "Other neoplasms
related to the meninges". This again changed in 2016 when the classi cation was updated.
Haemangiopericytomas and solitary brous tumours of the dura are considered di erent
manifestations of the same disease, listed under "mesenchymal, non-meningothelial tumours" as a
single entry. 

** It is important to note, when reading older literature, that in the WHO 2007
Case 22 Case 23
classi cation, in ltration into the brain parenchyma of an otherwise "benign" grade I tumour was
su cient to designate it a grade II tumour. As such, the incidence of grade II tumours increased to
~30% 11 .

Macroscopic
In general, there are two main macroscopic forms easily recognised in imaging studies:

globose: rounded, well de ned dural masses, likened to the appearance of a fried egg seen in
pro le (the most common presentation)
en plaque: extensive regions of dural thickening Case 24: sphenoid
Case 25
wing meningioma
The cut surface re ects the various histologies encountered, ranging from very soft to extremely
rm in brous or calci ed tumours. They are usually light tan in colouring, although again this will
depend on histological subtypes.

Radiographic features
In addition to histological variants, many of which have 'atypical' imaging appearances, a number of
'special examples' of meningiomas are best discussed separately. These include:

burnt out meningioma Case 26: MR Tractography

cystic meningiomas
intraosseous meningioma
intraventricular meningioma Imaging di erential diagnosis
optic nerve sheath meningioma
radiation-induced meningioma

The remainder of this section focuses on more typical imaging appearances of run-of-the-mill
meningiomas.

Plain radiograph
Plain lms no longer have a role in the diagnosis or management of meningiomas. Historically a
number of features were observed, including: Prostate metastasis to
sphenoid bone and …
enlarged meningeal artery grooves
hyperostosis or lytic regions
calci cation
displacement of calci ed pineal gland/choroid plexus due to mass e ect

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

non-contrast CT
60% slightly hyperdense to normal brain, the rest are more isodense
20-30% have some calci cation 8
post-contrast CT
72% brightly and homogeneously contrast enhance 8
malignant or cystic variants demonstrate more heterogeneity/less intense enhancement
hyperostosis (5%) 23
typical for meningiomas that abut the base of the skull
need to distinguish reactive hyperostosis from:
direct skull vault invasion by adjacent meningioma
primary intraosseous meningioma
enlargement of the paranasal sinuses (pneumosinus dilatans) has also been suggested to be
associated with anterior cranial fossa meningiomas 20
lytic/destructive regions are seen particularly in higher grade tumours but should make one
suspect alternative pathology (e.g. haemangiopericytoma or metastasis) ref

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 are typical, the
diagnosis can be made with a very high degree of certainty. In some instances, however, the
appearances are atypical and careful interpretation is needed to make a correct preoperative
diagnosis.

Meningiomas typically appear as extra-axial masses with a broad dural base. They are usually
homogeneous and well-circumscribed, although many variants are encountered. It seems that the
signal intensity of meningiomas on T2-weighted images correlates with the histological subtypes 29 .

Signal characteristics
Signal characteristics of typical meningiomas include:

T1
usually isointense to grey matter (60-90%) 3,8,13
hypointense to grey matter (10-40%): particularly brous, psammomatous variants
T1 C+ (Gd): usually intense and homogeneous enhancement
T2
usually isointense to grey matter (~50%) 3,8,13
hyperintense to grey matter (35-40%)
usually correlates with a soft texture and hypervascular tumours 13
seen in microcystic, secretory, cartilaginous (metaplastic) chordoid and angiomatous
variants 12
hypointense to grey matter (10-15%): compared to grey matter and usually correlates with
harder texture and more brous and calci ed contents
DWI/ADC: atypical and malignant subtypes may show greater than expected restricted di usion
although recent work suggests that this is not useful in prospectively predicting histological
grade 15,16
MR spectroscopy: usually does not play a signi cant 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 signi cantly reduced N-acetylaspartate (NAA): non-neuronal origin
absent or signi cantly reduced creatine (Cr)
MR perfusion: good correlation between volume transfer constant (k-trans) and histological
grade 28
MR tractography: allows the identi cation of white matter tracts adjacent to the meningioma
this may aid in preoperative planning for meningioma resection by allowing planning of a
safer access route that would result in less residual functional iatrogenic de cits 30

Helpful imaging signs

A number of helpful imaging signs have been described, including:

CSF cleft sign, which is not speci c for meningioma, but helps establish the mass to be extra-
axial; loss of this can be seen in grade II and grade III which may suggest brain parenchyma
invasion
dural tail is seen in 60-72% 2  (note that a dural tail is also seen in other processes)
sunburst or spoke-wheel appearance of the vessels
white matter buckling sign
arterial narrowing
typically seen in meningiomas which encase arteries
useful sign in parasellar tumours, in distinguishing a meningioma from a pituitary
macroadenoma; the latter typically does not narrow vessels

Oedema
More than half of the meningiomas demonstrate a variable amount of vasogenic oedema in
adjacent brain parenchyma 24 . Correlation between age, gender, tumour size, rapid growth, location
(convexity and parasagittal > elsewhere), histologic type, and invasion in the case of malignant
meningiomas have been suggested in literature but not yet con rmed. Although in general, the
presence of severe adjacent oedema is considered more compatible with aggressive meningiomas,
in some histologically benign types such as secretory type, oedema can be disproportionately larger
than the small tumour size.

The underlying mechanism is most likely multifactorial however it has been shown that there is a
strong association between the presence and severity of the peritumoral vasogenic oedema (i.e.
oedema index) and expression of the vascular endothelial growth factor (VEGF) or expression of CEA
and CK 17,25 .

List of some of the proposed underlying mechanisms are:

venous stasis/occlusion/thrombosis
compressive ischaemia
aggressive growth/invasion
parasitisation of pial vessels
histologic subtype: secretory meningioma 25
vascular endothelial growth factor (VEGF): produced within the meningioma that enters the
adjacent parenchyma
expression of CEA and CK

Angiography (DSA)
Catheter angiography is rarely now of diagnostic use but rather is performed for preoperative
embolisation to reduce intraoperative blood loss and alleviate resection of a tumour. This is
especially useful for skull base tumours, or those thought to be particularly vascular (e.g. microcystic
variants or those with very large vessels). Particles are favoured typically 7-9 days prior to surgery
although they are not free of complication, particularly one study showed a high prevalence of
complications associated with particles smaller than 45-150 μm, so risks and bene ts should be
thoroughly assessed 26 .

Meningiomas can have a dual blood supply. The majority of tumours are predominantly supplied by
meningeal vessels; these are responsible for the sunburst or spoke-wheel pattern observed on
MRI/DSA. Some tumours also have a signi cant pial supply to the periphery of a tumour.

A well known angiographic sign of meningiomas is the mother-in-law sign, in which the tumour
contrast blush "comes early, stays late, and is very dense".

Treatment and prognosis

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

The Simpson grade correlates the degree of surgical resection completeness with symptomatic
recurrence.

Recurrence rate varies with grade and length of follow-up 8,21

grade I = 7-25%
grade II = 29-52%
grade III = 50-94%

Metastatic disease is rare but has been reported 8 .

History and etymology

The term "meningioma" was rst introduced by Harvey Cushing, a renowned American
neurosurgeon, in 1922 9,23 .

Di erential diagnosis
The di erential diagnosis generally includes other dural masses as well as some location-speci c
entities.

The main dural masses to consider include:

haemangiopericytoma
more aggressive often destroying bone
extensive peripheral vascularity
more microlobulation
dural metastases (e.g. breast cancer)
for other less common di erentials see dural masses

Speci c location di erentials include:

cerebellopontine angle
acoustic schwannoma
pituitary region
pituitary macroadenoma
craniopharyngioma
base of the skull
hypertrophic pachymeningitis
extramedullary haematopoiesis
chondrosarcoma
chordoma

In the setting of hyperostosis consider:

Paget's disease
brous dysplasia
sclerotic metastases (e.g. prostate and breast carcinoma)

Quiz questions

References

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Dural masses
dural masses
meningioma
grading and histological variants
grade I [+]
grade II [+]
grade III [+]
imaging signs
CSF cleft sign
dural tail
ginkgo leaf sign (spinal meningioma)
mother in law sign
spoke wheel sign
sunburst sign
white matter buckling sign
variants
cystic meningioma
en plaque meningioma
radiation-induced meningioma
burnt out meningioma
by location
primary intradural meningioma [+]
primary extradural meningiomas [+]
Simpson grade (of resection)
haemangiopericytoma
solitary brous tumour of the dura
primary dural lymphoma
Rosai-Dorfman disease
EBV-associated smooth muscle tumour
meningeal melanocytoma
primary meningeal malignant melanoma
Erdheim-Chester disease
sarcoidosis
dural metastases
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