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Meningioma

Dr Bruno Di Muzio and Dr Frank Gaillard et al.

Meningiomas are extra-axial tumours and represent the most common tumour of the
meninges. They are a non-glial neoplasm that originates from the arachnoid cap cells of
the meninges. They are typically benign with a low recurrence rate but rarely can be
malignant.
Typical meningiomas appear as dural based masses isointense to grey matter on both T1 and
T2 weighted imaging, and demonstrate vivid contrast enhancement on both MRI and
CT. There are, however, many variants some of which can vary dramatically in their imaging
appearance.
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. Atypical and malignant meningiomas are slightly more common in males. They are
uncommon in patients before the age of 40 and should raise suspicion ofneurofibromatosis
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 with neurofibromatosis type II (NF2) (Merlin
gene on Chromosome 22). Additionally meningiomas demonstrate oestrogen and
progesterone 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
Oedema may be present associated to some meningiomas and the underlying mechanisms for
this is related to:

venous stasis/occlusion/thrombosis

compressive ischaemia

aggressive growth/invasion

parasitisation or pial vessels

VEGF producing lesion


Macroscopic
In general, there are two main macroscopic forms easily recognized on image studies:

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

Variants

intraosseous meningioma: sclerotic or lucent

rarely degeneration into:


o

cystic meningioma

osteoblastic meningioma

chondromatoid meningioma

meningioma with sarcomatous degeneration

meningioma with fatty degeneration

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
o

clear cell meningioma: have up to a 60% recurrence rate and occur in


younger patients 6
malignant meningioma (WHO III)

uncommon accounting for only 2.4% of all meningiomas 4 and


demonstrates 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


o
o

more frequently multiple, and typically occur ~35 years after radiotherapy
meningiomas are a much more frequent complication of radiotherapy
compared to sarcomas or gliomas
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


o

intraventricular meningioma (choroid plexus)

optic nerve meningioma

pineal gland

spinal: especially thoracic (see spinal meningioma)

<1% "extradural"
o

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, the rest are more isodense

20-30% have some calcification 8

72% brightly and homogeneously contrast enhance 8, less frequent in malignant or


cystic variants

hyperostosis
o

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: particularly in higher grade lesions

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
o

isointense: ~60-90% 3,8, 13

somewhat hypointense: 10-40% compared to grey matter

T1 C+ (Gd): usually intense and homogeneous enhancement

T2
o

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
o

12

hypointense: 10-15% compared to grey matter and usually correlates with


harder texture and more fibrous and calcified contents

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

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

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

o
origin
o

absent or significantly reduced creatine (Cr)


MR perfusion: it has good correlation between volume transfer constant (ktrans) and histological grade

Helpful signs include:

CSF vascular cleft sign, which is not specific 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 seen in 60-72% 2 (note that a dural tail is also seen in other processes)

sunburst or spokewheel appearance of the vessels (case 9)

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), and invasion in the case of malignant meningiomas.
Angiography (DSA)

mother-in-law sign: "comes early, stays late, very dense", tumour blush

dual blood supply from both


o

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, and generally includes otherdural
masses:

cerebellopontine angle
o

acoustic schwannoma
parasellar region

pituitary macroadenoma

craniopharyngioma
elsewhere

haemangiopericytoma

granuloma

sarcoidosis

tuberculosis

idiopathic hypertrophic pachymeningitis

extramedullary haematopoeisis

chondrosarcoma

chordoma

In the setting of hyperostosis consider:

Paget's disease

fibrous dysplasia
In the setting of lucent intraosseous meningioma the differential is essentially that of
a solitary lucent lesion of the skull.