Meningioma

Karakteristik dengan Hyperostosis

 Floor of the middle fossa is an area frequently involved --> removal of the
hyperostotic bone provides a more complete resection and leads to a lower
rate of recurrence
Meningiomas are the most common benign intracranial neoplasms, accounting for 13
to 26% of all intracranial tumors

Meninges
 Dura Mater
 Arachnoid
 Pia Mater
 Arachnoid Villi/Granulations
  Meningiomas accounted for 33.8% of all primary brain and central nervous
system (CNS) tumors
 Meningiomas account for ~20% of all intracranial tumors in males and 38% in
females
Risk Factors
 Ionizing Radiation
 primary environmental risk factor identified for meningioma is exposure to
ionizing radiation (IR), with risks from 6- to 10-fold reported
 Hormones
 association between hormones and meningioma risk has been suggested by
several findings, including the increased incidence of the disease in women
versus men (2:1); the presence of estrogen, progesterone, and androgen
receptors on some meningiomas
 Head Trauma
 Since the time of Harvey Cushing, head trauma has been suggested as a risk
factor for meningioma, although the results across studies are not consistent
 Cell Phone Use
 The question of whether cell phone use is related to meningioma risk remains
of great interest to the general public. At least 10 studies have examined the
association between cell phone use and tumors of the brain
 Association with breast cancer
 An association between breast cancer and meningioma has been examined in
several studies.2,4,22
 Several explanations have been proposed for this association, including the
presence of common risk factors, such as endogenous and exogenous
 hormones as well as shared genetic predisposition, including variants in
DNA repair polymorphisms
 Industry/Occupation/Diet/Allergy
 Family History of Meningioma

Pathology of Meningioma
 Meningiomas are slowly growing neoplasms thought to arise from meningothelial
cells found within arachnoid granulations. Concentrated in the walls of the major
venous sinuses, these structures, which contain “arachnoid cap cells,” account for
the dural localization of most meningiomas within the cranium and spinal cord
Localization
 The majority of meningiomas are supratentorial, with a large number located
along the convexities. Approximately 17 to 25% occur in a frontobasal location;
however, only about 10% occur in the posterior fossa
 frontobasal region, the olfactory grooves, tuberculum sellae and parasellar region,
and the petrous bone are preferred sites. Approximately 5% occur along the
cerebellar convexity, 2 to 4% at the tentorium cerebelli, and 2 to 4% within the
cerebellopontine angle
Histopatology
 WHO Grade I
 Meningothelial
 Fibrous
 Transitional
 Microcytic
 Psammomatous
 Secretory
 Angiomatous
 Metaplastic
 Lymphoplasmacyte-Rich
WHO Grade II
 Atypical Meningioma
 On microscopic examination, atypical meningiomas deviate from their benign
counterparts by the presence of increased mitotic activity [four or more mitoses
per 10 high power fields (HPFs)], or three or more of the following changes:
increased cellularity, small cell formation, prominent nucleoli, sheetlike growth,
and areas of spontaneous necrosis
 Clear Cell
 Chordoid
WHO Grade III
 Anaplastic
 This malignant variant can be recognized by its greater cellularity, malignant
cytology, and increased mitotic activity, usually more than 20 mitotic figures per 10
HPF. Necrosis is common in atypical and malignant forms of meningioma.
Fortunately, their incidence is relatively low, ranging from 0.9 to 10.6% in different
series, with an overall mean representation of 2.8% of meningiomas.
 Rhabdoid
 Rhabdoid meningiomas are uncommon
 Papillary

Molecular Biology of Meningiomas: Tumorigenesis and Growth

 Meningiomas are graded as benign (~92% of meningiomas), atypical (6%), or
anaplastic/malignant (4%), based on histological characteristics
 Chromosomal alteration in meningioma
 Chromosome 22q: NF2 Gene and the Gene Protein Product, Schwannomin/merlin
Modern Imaging Techniques for Meningiomas
 Computed Tomography
 CT has a place in the diagnosis of meningioma because it is superior in
demonstrating the effects of this neoplasm on adjacent bone, specifically osseous
destruction or hyperostosis, and is more sensitive in detecting psammomatous
calcifications in the tumor (seen grossly in ~25% of meningiomas)
 Benign meningiomas typically appear as rounded or elongated extraaxial masses
that demonstrate a broad attachment to the dura. On CT, they are usually isodense
but can occasionally be hyperdense or slightly hypodense compared with cerebrum
 Their extraaxial nature is suggested by a sharp interface with displaced brain
parenchyma, the presence of a cerebrospinal fluid attenuation cleft and intense
enhancement.
 Hyperostosis of adjacent skull is highly suggestive of benign meningioma and is
best demonstrated by CT, windowed on bone algorithm, as cortical thickening and
hyperdensity. Hyperostosis typically indicates infiltration of bone by meningioma
 Magnetic Resonance Imaging
 Approximately 85 to 90% of meningiomas have typical features, including an
extraaxial mass with signal intensity isointense to cortex on T1 and T2 MRI
sequences, avid homogeneous enhancement following administration of
gadolinium contrast, and an enhancing “dural tail”, which reflects neoplastic dural
infiltration or reactive vascularity (or both) draining into the adjacent dura

 Advanced Imaging
 Diffusion Magnetic Resonance Imaging
 With diffusion-weighted imaging (DWI), each image voxel (three dimensional)
has an image intensity that reflects a single best measurement of the rate of
microscopic water motion at that location. Reduced water diffusivity (Fig.
13.12A) has been correlated with more aggressive tumor behavior and is seen
 with atypical/malignant meningiomas, high cellular density, and recurrence.14
The apparent diffusion coefficient (ADC) map, a calculated image from the
DWI image, shows the average diffusion that water molecules have in each
voxel. This parameter is calculated from all the diffusion-weighted images. A
decrease in ADC values (Fig. 13.12B) at followup of a benign meningioma
should raise suspicion for dedifferentiation to higher tumor grade.
 Perfusion

Surgical Treatment of Intracranial Meningiomas by Site

 mostly benign (1% malignant), slow-growing, non-infiltrative

 common locations:
o parasagittal(20.8%)
o convexity(15.2%)
o TSM(12.8%)
 o sphenoid wing(11.9%)
o falx(8%)
 presentation: middle aged, symptoms of increased ICP, focal symptoms
depend on location
 diagnosis: MRI, CT with contrast (see Figure 5)
 therapy
o conservative management for slow-growing lesions
o surgery is treatment of choice (curative if complete resection)
o radiotherapy – ineffective
o prognosis: > 90% 5-yr survival

Meningioma Location

CPA
Falx vs Parasagital Meningioma
Falx Parasagital
 Attachment falx  Attachment trough Sagital sinus
 Usually no midline shift and convexity
 Usually midline shift

Simpson grading system for removal of meningiomas

Complete removal

Partial removal

Meningioma
- Tumbuh lambat, ekstraaksial, biasa jinak.
- Asal : arachnoid cap cells
- 32% meningioma yang ditemukan insidentil tidak tumbuh dalam 3 tahun 
0.
- Paling sering = Falx, konveksitas, tulang sphenoid.
- 8% multipel, seting pada NF ekstraaksial
- Neoplasma primer intrakranial paling sering (14,3-19%)
- Puncak insidens : usia 45 tahun.
- ♀/♂ : 1,8 : 1 , > 60 th

Sphenoid wing/ridge meningioma, DD/ Temporal base m

1. Lateral (pterional) sifat dan Tx mirip meningioma konveksitas.
2. 1/3 medial (alar)
3. Medial (dinoidal) membungkus ICA & MCA & N. Cranial di regio FOS & N
optic, dapat menekan brainstem, removal  sulit.
4. Nempel ke SSS ! / Falx meningioma : - nempel ke konveksitas falk, sinos
Sampai 50% menginvasi SSS (ARAH AP) biasa  menyebabkan midline
shift
1. Anterior (ethmoidal place to coronal suture) 33%
Gejala : sakit kepala, perubahan status, mental.
2. Middle (antara sutura koronaria & lambdoid) 50%
Gejala : Kejang Jacksonian & monoplegi progresif
3. Posterior (sutura lambdoid – torcular herophili) 20%.
Gejala : nyeri kepala, gejala visual, kejang fokal, perubahan status
mental.
Parasagital meningioma dapat berasal dari level motor = manifestasi inisial =
contralateral foot drop, lower ext contra lateral seizure 1/3 kasus, frontal lobe
syndr post 1/3 : hemianopsia homonim

Parasagittal Meningioma invasion

 Type I = nempel ke dinding lateral sinus
Type II = Invasi ke resesus lateral
Type III = Invasi ke dinding lateral
Type IV = Invasi ke lateral wall and roof.
Type V = Total sinus occ lost on contralateral wall
Spaired
Type VI = total sinus occlusion, invasion of all walls
Sistem grading invasi meningioma ke SSS (Sindov MP, 2006)
1. Olfactory groove meningioma
2. Mental status change
3. Inkontinensia urin
4. Menekan apparatus optik  ggg visual  menekan optic nerve
Lesi besar 
5. Menekan fornix  short term memory loss
6. Kejang
- Morbiditas, mortalitas dan kesulitan total removal meningkat signifikan
pada tumor berukuran > 3 cm.
- Pre Op. MRA CTA atau angiogram berguna untuk mengetahui lokasi ACA
terhadap tumor.
- 70-80% olfactory groove meningioma mendapat supply dari A. ethmoidal
anterior, yang biasanya tidak dapat diembolisasi karena risiko mengenai
A-Ophtalmic (kebutaan)

Batas frontal / fossa anterior :

Anterior : Posterior dinding sinus frontal.
Posterior : Lesser wing of the sphenoid dan proc. Clinoid anterior
Lateral : orbital place of the frontal bone
Floor : atap civitas nasal dan sinus ethmoid di medical
Bila tumor berukuran > 3 cm : morbiditas, mortalitas , sulit total removal.
2. Planum sphenoidale meningioma
- berasal dari flat part of sphenoid bone, anterior dari sulkus chiasmatic,
di bagian posterior anterior cranial fossa.
Gambar:

3. Tuberculum sella meningioma (TSM)

- asal : 2 cm posterior dari alfactory groove meningioma
- tubercullum sellae adalah penonjolan tulang antara sulcus, chiasmatic
dan sella tursica.
- Termasuk dalam fassa media (planum sphenoid  f.ant)
- TSM sering menyebabkan visual loss
(Chiasmal syndrome = Primary optic atiophy + bilateral hemianopsia)
Patologi : 4 variabel histopatologi :
1. Grade
2. Subtipe histologis
3. Proliferation indices
4. Brain invasion
Frontal base meningioma : 1-4
Klasifikasi meningioma, WHO, 2000
1. Meningioma dengan resiko rekurensi rendah/tidak agresif (WHO gr I)
A = Meningiothelial or meningotheliomatous, (syncytial )
The most common
Istilah angiomatous untuk meningotheliomatous bervariasi u/ closed
packed blood vessels.
B = Fibrous / fibroblastic
Konsistensi lebih kenyal dari meningotheliomatous/transision
C = Transitional = diantara meningotheliomatous dan fibrous kadang ada
Kalsifikasi (psammoma bodies)
D = Psammomatous = calcified meningothelial whorls
E = Angiomatous
F = Microcystic AKA “humid” or vasiculated meningioma
G = Secretory
H = Lymphoplasmacyte - rich
2. Meningioma with greater recurrence risk and/or agresif
A = Atypical meningioma
B = Rhabdoid meningioma : usually have malignant agresif features
C = Malignant meningiomas AKA anaplastic, papillary or sarcomatous,
morecommon in younger pk
Proliferation indices
- Tidak bisa digunakan tunggal untuk grading berguna untuk prognosis yang
diperiksa = Ki – 67.

K1 – 67 Proliferation index in meningioma

Description dan WHO Mean ki-67 index Recurrence rate
grade
Common meningioma (gr I) 0,17 % 9%
Atypical meningioma (gr II) 2,1% 29 %
Anaplastic meningioma (gr 11% 50%
III)
Brain invasion
Adanya brain invasion meningkatkan rekurensi seperti rekurensi pada atypical
meningioma (not anaplastic) tapi bukan indikator malignancy.
Metastase
Sangat jarang metastase keluar CNS
Kebanyakan angioblastik / ganas.
Most common site : lung, liver, lymph node, heart
Klasifikasi WHO untuk meningioma
Grade 1 : Meningotelial
Fibrous (fibroblastic)
Transitional (mixed)
Psammomatous
Angiomatous
Microcystic
Lymphoplasmacyte rich
Metaplastic
Grade II : Chordoid
Clear cell (intracranial)
Atypical
Grade III : Papilary
Rabdoid
Anaplastic
DD meningioma :
1. Multiple meningioma : suggest NF tipe 2
2. Pleomorphic xanthoasrocytoma (PxA)
Mtrip meningioma karena lokasi lebih ke perifer dan biasa ada dural tail.
3. Rossai – Dorfman disease :
Connective tissue disorder with sinus histiocystosis & massive painless
lymphadenopathy.
Biasanya dewasa muda.
Radiologi
MRI : Iso-intense pada T1 dan T2, menyengat dengan Gd homogen
kalsifikasi bisa positive (+)
Infiltrasi duris/reactive vasolianty dural tail : common dapat memberi informasi
keterlibatan sinus.
CT : Menyengat kuat, homogen dengan kontras karena mendapat supply dari
ECA (no BBB), Ca prostat jarang metastase ke otak. Biasanya ke tulang,
dapat menimbulkan hiperostosis pada skull
Angiografi : comes early, stays late, early in arterial phase, persists beyond
venous phase.
Karakteristik meningioma : ada feeder dari ECA, kecuali :
- Olfactory groove  large branches of ophtalmic arteries.
- Parasellar  ICA
- Tentorial  a bernasconi cassinari AKA
Artery of tentorium AKA
Itarian artery enlarged
- Embolisasi pre OP :
Mengurangi vaskolarisasi tumor sebelum CTR.
Waktu pembedahan : kontroversial.
Dikatakan menunggu 7-10 hari agar tumor dapat nekrosis
Meningioma
5 year survival rate : 91,3%
Rekurensi :
- gross total removal : 11-15% kasus
Incomplete : 29%
- 5 years reccurence rate pada inkomplete : 37-85%
Simpson grad mg
I = Complete removal (macroscopic) dan extension of dural attachment
& abnormal bone (termasuk reseksi sinus bila terlibat)
II = Complete removal dengan koagulasi endotermal dura yang terlibat
III = Complete reserval tanpa reseksi/koagulasi perlekatan dura/ekstensi
ekstradura
IV = Partial removal leaving tumor in situ
V = Dekompresi simple (± biopsi)
Pada operasi SOM, tulang apa yang didrill ?
- Orbital wall sebelah lateral, temporal, sphenoid, alar, dinoid.
Gangguan visus  karena penekanan pada optic nerve