Ch.

62 Meningioma
Ch. 63 Meningeal
Hemangiopericytoma
Ch. 64 Meningeal Sarcoma


Ch. 62 Meningioma
Embryology and histology
Three distinct layer of meninges

Dura mater (pachymeninges)
Arachnoid
Pia mater (leptomeninges)

Gestation 22-24 days: monocellular layer (from neural crest)
subsequently pia mater
33-41 days: multiple layer by mesenchymal cells
arachnoid and dura mater

Arachnoid: arachnoid barrier cells & arachnoid trabecular cells
~ CSF reabsorption (arachnoid villi)

Arachnoid cap cell : arachnoid villi venous sinus
protrusion ,venous endothelium

Meniogiomas grouped by likelihood of
recurrence and grade
WHO Gr. I
Meningothelial MNG Psammomatous M
Fibrous MNG Angiomatous M
Transitional MNG. Microcystic M

WHO Gr. II
Atypical MNG
Clear cell MNG
Chordoid MNG

WHO Gr. III
Rhabdoid MNG
Papillary MNG
Anaplastic MNG (malignant)


Pathology
;
convexity, 35%
parasagittal, 20%
sphenoid ridge, 20%
intraventricular, 5%.

;
psammoma bodies
mineralized whorl - calcium apatite + collagen
intranuclear cytoplasmic pseudoinclusion
Orphan Annies eye nuclei


Pathology
Atypical MNG:

High recurrence rate, aggressive growth
High mitosis (10 HPF 4 )

Increased cellularity
Small cells with a high nucleus-to-cytoplasm ratio
prominent nucleoli
Uninterrupted patternless or sheet-like growth
Foci of spontaneous or geographic necrosis


Pathology
Anaplastic MNG:

more severe MNG than atypical MNG
malignant cytology,
high mitotic index (20 /10 HPF )

Immunohistochemistry; epithelial membrane antigen (EMA) (+) of
80%
S-100 staining: variable
Anti-leu 7: schwannoma (+) / MNG(-) D/Dxd
GFAP: almost (-)
Nonhistologic diagnosis
MRS and ion exchange chromatography.
Hyperostotic bone diploe haversian canals
.
Aggressive behavior of meningioma
Hypercellularity, loss of architecture,
Nuclear pleomorphism
Increased mitotic indexes, focal necrosis,
hypervascularity,
Hemosiderin deposition, small cell formation

BUdR (Bromodeoxyuridine) labeling technique
: mitosis S phase cell

Ki-67: previous RT (+)

TGF-a; malignancy .
Aggressive behavior of meningioma

E-cadherin;
choroids plexus papillomas & meningioma
benign MNG recurrent MNG (+)
malignant MNG (-)

SPARC; invasive tumor

Progesterone receptors(-) ; poor outcome



1~16% of MNG
60-90%:

Associated with Neurofibromatosis type II
Edge CSF seeding
Intraosseous MNG ; mainly cranial bone
Extraneuraxial MNG; orbit, paranasal sinus, nasopharynx, skin..

MNG 20%
3/4

- 1) slow growing tumor
- 2) increased vascularity of MNG
- 3) peculiar microenvironment

Multiple meningiomas
?
Epidemiology
22%
2.3 cases / 10 , 5.5/10
(autopsy )
: (1.5:3.1 per 10)

autopsy case MNG glioma
(40%)

MNG
70 . ,

- less aggressive surgical approach in the elderly
- failure to realize

Meningioma
Very rare: 1-4%

;
.
infancy 71%
unusual sites
intraventricular MNG (11%)
multiple(23%), cystic component(23%)
associated with NF
dura attachment (13%)
Etiology
1.Trauma : 1922 Cushing mentioned .
meningioma

2. Virus : association with IMV

3. Radiation :


4. others : other disease, breast cancer,
venous thrombosis etc


Genetic aspects of Meningioma
Chromosome 22 long arm(22q)

Meningioma 50%

- NF2 tumor suppressor gene
- SIS oncogene PDGF beta locus MNG
- LOH for 1p; anaplastic MNG, atypical MNG, common type
- INI1 gene; rhabdoid MNG

Most frequent chromosomal loss in malignant and atypical tumors
- 14q

NF2 gene- merlin= schwannomin
DAL1 60% of sporadic MNG

Meningioma & Receptors
progesterone receptor
- cytoplasm nucleus .

somatostatin receptor(ss2A), androgen, glucocorticoid
receptors.D1 receptor(but not D2), prostaglandin E2,
EGF(epidermal growth factor), fibroblast growth factor,
PDGF,prolactin receptor, CEA receptor
Radiology
Plain x-ray film:
1) hyperostosis 2) increased vascular marking
3) calcification


CT: iso or slight hyperdense / homogeneous enhance.
sharply marginated, broadly based against a bony structure or
dura.hyperostosis,
15% cystic change, necrosis(+)

Dural tail sign:

tumor cell
connective tissue vascular tissue .


Radiology
MRI: T1WI- isointense(60%), mild hypointense(30%)
T2WI- iso (50%), hyperintense (40%)
T2WI high signal water content
meningothelial MNG, vascular MNG, aggressive MNG
.

Angiography: to assess the vascularity and vascular supply of
tumor, embolization , tumor encroachment on vascular
structure

Somatostatin receptor scintigraphy (SRS) using 111Inoctreotide:
meningioma sensitive
residual tumor
postoperative change .

PET using FDG:
aggressive MNG benign MNG
D/Dx: sarcoidosis, solitary fibrous tumor of meninges
(CD34+, vimentin+, EMA-, S100-)

Surgical therapy & tumor recurrence
Simpson grade (recurrence , grade I 10%)
Dural margin 2cm grade 0
( 0% : Al-Mefty)

, meningioma .
sphenoid wing meningioma, en plaque MNG
.(>20%)

Useful predictor of recurrence
<Yamasaki & colleagues>
high levels of expression of VEGF, high MIB-1 labeling index. PCNA
and Ki-67 MNG

<Nakasu>
shape of tumor tumor .,
mushrooming and lobulated meningiomas round MNG
.

Nonsurgical therapy for Meningiomas
Radiation therapy
<by Guthrie & asso.>

) malignant meningioma ,
) ,
) multiple recurrent tumor ,
)

Hormonal treatment
Tamoxifen (estrogen antagonist), bromocriptine,
mifepristone(RU 486), gestrinone hormone, hydroxyurea,
interferon alpha-2B, trapidil ..
Convexity Meningioma
15%

Most common presentation:
seizure,
incidental finding

1957, Simpsons grade
additional 2cm margin of dura
(grade 0) by Al-Mefty

blood supply
by middle meningeal branches
(typical sunburst pattern)


Parasagittal Meningiomas
<Cushing & Eisenhardt>
no brain tissue between tumor and sup. sagittal sinus

tumor superior sagittal sinus involve


Ligation of sinus venous infarction
anterior 1/3

sinus collateral
venous channel .
sinus repair
Falcine meningiomas
Three types
) Anterior type; crista galli coronal suture
1/3
) Middle type; coronal suture lambdoid
suture 1/3
) Posterior type; lambdoid suture torcula
1/3

<by Yasargil> 2 types
) Outer type; frontal, central parietal,
occipital region falx
) Inner type; origin from inferior sagittal sinus

Intraventricular meningiomas
Choroids plexus tela choroidea arachnoid cells
.

1%, 90% lateral ventricle trigone
blood supply by Anterior or Posterior choroidal artery


cortical dysfunction .

corpus callosum midline incision :
right homonymous hemianopsia
complete splenial section alexia without agraphia


temporal gyrus posterior paramedian parieto-occipital
approach .
Tentorial meningiomas
Petroclival meningioma: brain stem one layer arachnoid
adhesion .

tentorial meningioma: interpeduncular, crural , ambient cistern
tentorial edge multiple layer of arachnoid


venous system
. vein of Labbe, basal temporal veins,
superior petrosal sinus, tentorium, sigmoid sinus, jugular bulb
.

Tentorial meningiomas
Anterior to mid-medial
incisural ring;
zygomatic extended middle
fossa approach

Middle to posterior part of inner
ring of tentorium; petrosal
approach

Falcotetorial lesion;
posterior interhemispheric
transtentorial approach or
supracerebellar infratentorial
approach,
supra- infratentorial approach

Olfactory Groove meningiomas
MRA: anterior cerebral artery
CT : calcification, skull base invasion

anterior ethmoidal artery
ethmoid & nasal cavity extracranial extension
- mucosa exenteration fat sinus dural
graft, pericranial flap frontal fossa

surgical approach;
() Unilateral pterional approach
() subfrontal approach
() supraorbital approach

Sphenoid wing &
clinoidal meningiomas
clinoidal, middle and lateral sphenoid wing.
sphenoid bone hyperostosis diffuse tumor invasion
proptosis cranial neuropathy .

Surgical tactics; greater wing of sphenoid wing, clinoid, superior and
lateral orbital wall repair.

Middle 1/3 of sphenoid wing meningioma: cranio-orbital
zygomatic craniotomy
Clinoid meningioma by Al-Mefty
Group I; tumor carotid cistern proximal portion
ACP . ICA interfacing arachnoid membrane
adventitia adhesion

Group II: ACP supero or lateral aspect .
carotid cistern, sylvian cistern arachnoidal membrane arterial
adventitia . Group I, II optic
chiasm chiasmatic cistern
.

Group III: optic foramen optic canal ACP tip
extension. optic nerve compression
. - chiasmatic cistern proximal portion
optic nerve arachnoid
.



Tuberculum Sellae meningiomas
5-10% .
30 : > X3
Primary optic atrophy with bitemporal field defect

Fundoscopic finding: primary optic atrophy(+)

Olfactory meningioma anosmia

. optic canal extension.

Diaphragma sellae meningioma
retrochiasmatically hypothalamic dysfunction


Tuberculum Sellae meningiomas
Type A:
upper leaf of diaphragma sellae, stalk


Type B:
upper leaf of diaphragma selle, stalk


Type C:
inferior leaf of diaphragma sellae

Cavernous sinus meningiomas
Approach: cranio-orbital zygomatic approach proximal
ICA control petrous bone ICA
( Glasscocks triangle)

Entry medial or lateral triangle
optic nerve sheath dura propria canal
opening distal dural ring oculomotor
trigone proximal dural ring .
ICA mobilize.

Lateral entry cavernous sinus lateral wall outer
dural layer peel away
Meningiomas of the optic nerve and
orbit
Optic nerve sheath . orbit meningioma
superior orbital rim, roof, inferior orbit .
anterior clinoidal meningioma optic foramen
.

Typical symptoms of orbital meningioma:
progressive, painless visual loss& proptosis


Posterior fossa meningiomas
Trigeminal nerve petroclival
meningioma CPA meningioma
petroclival meningioma .

CPA meningioma
petroclival meningioma
Jugular foramen meningioma
Foramen magnum meningioma
CPA meningiomas
4
th
& 5
th
nerve : tumor superior & lateral p.
6
th
nerve : tumor anterior portion
7
th
& 8
th
nerve : tumor anterior portion
9
th
&10
th
nerve : tumor inferior portion

Retrosigmoid approach
: tumor capsule central debulking and
devascularization .
for large tumor
: cranial nerves superomedially SCA,
medially AICA, inferomedially PICA


Petroclival meningiomas
3 type; Clival MNG
superior 2/3 of clivus, brainstem
Petroclival MNG
sup. 2/3 of clivus, 5th nerve
Sphenopetroclival MNG
sphenoid formcavernous sinus lateral wall
involve.
Surgical approach:

posterior petrosal approach
(presigmoid transtentorial)
Anterior petrosal approach
Combined approach


Jugular foramen meningiomas
Jugular bulb arachnoid cells .
Jugular bulb -> lower cranial nerve deficit

D/Dx: glomus jugulare tumors, neuromas

Jugular bulb patency dominance
Jugular bulb transjugular approach,
suprajugular, retrojugular approach .



Foramen Magnum meningiomas
2 types types;

Ventral type- clivus lower 1/3 basal groove

spinocranial type upper cervical area .
spinal cord
cerebellomedullary cistern growth

Symptom triad:
cervical pain, motor and sensory deficits(
spastic quadriplegia ), cold & clumsy hands with
intrinsic hand atrophy.

Lateral or posterior FM MNG: SOC
ventral type :transcondylar approach


Surgical approaches for base of the skull meningiomas
Cranio-orbital zygomatic approach
Patient position: supine,
30-40 degrees head rotation
Technique:
Bicoronal incision
superficial & deep fascia of
temporalis m.
zygoma cutting, temporalis
m inferior retraction, burr
hole, orbital roof cutting
, lateral wall and roof of
orbit removal .


extradural removal of anterior clinoid
process, subclinoid ICA ,
petrous ICA


Zygomatic extended middle fossa approach
Standard subtemporal approach
+ transzygomatic approach

Technique: interfascial
approach,removal of zygomatic
arch, inferior retraction of
temporalis m. removal of bone
flap

Anterior petrosal approach: extradural approach, 5th
nerve 2nd, 3rd branch .
middle meningeal artery foramen spinosum
coagulation and cutting, greater petrosal nerve
.

Petrosal approach & extended petrosal
approach
Petrosal approach : Perimesencephalic or
peripontine structures .
retrosigmoid approach cerebellum,
temporal lobe retraction neural structures, otologic
strucutures, major veins
Position: 30 degrees flexion, 50 degrees rotation
Petrosal approach & extended petrosal
approach
Technique:

Transverse sinus - 2 burr hole



Temporoparietal craniotomy
Complete mastoidectomy

Skeletonization of sigmoid sinus
Sinodural angle and Citellis angle
Superior petrosal sinus
Drill the superficial & deep mastoid air cells
Facial canal, semicircular canal
Opening posterior fossa dura
Temporal fossa dura open
Superior petrosal sinus coagulation & ligation
Dural incision pyramid incisura
tentorial edge 4th nerve
.

Transcondylar approach
first, SCM m occipital bone
2nd muscle layer: splenius capitis, longissimus capitis,
semispinalis muscles SCM inferomedially
..
3rd muscle layer: 2 triangle
superior suboccipital triangle (rectus capitis posterior
major, obliquus capitis superior, obliquus capitis inferior m)
venous compartment, horizontal part of vertebral
artery, C1 nerve .
Inferior suboccipital triangle ; obliquus capitis inferior,
semispinalis cervicis, longissimus cervicis m.
vertebral part of vertebral a. surrounding
venous plexus, C2 nerve .



Ch. 63 Meningeal
Hemangiopericytoma
Chapter 63.
Meningeal Hemangiopericytoma
sarcoma
meningeal capillary pericytes precursor cells
with angioblastic tendency
.

angioblastic meningioma WHO
classification meningeal hemangiopericytoma
.


Gross & microscopic pathology
Grossly, firm and lobulated, pink-gray~red color

Brain invasion en plaque
calcification .

staghorn capillary(+)
microcysts, necrosis, papillary architecture(+) whorls, psammoma
body .
Reticulin cell .

Marker factor XIIIa meningioma
vimentin, HLD-DR, CD34, Leu-7, S-100
protein positive.
fVIII-RA (-), EMA (-), GFAP(-)


Molecular characteristics &
biologic behavior
Chromosome 12q13 . MDM2, CDK4, CHOP/153

(meningioma 12q13 MNG
NF2 tumor suppressor gene
hemangiopericytoma .)

MIB-1, Ki-67, DNA ploidy .

Incidence
Meningioma 2-4% . 10%
meningioma .

Clinical findings

Meningioma (56-73%)
40 , MNG posterior fossa(15%), spine
(15%) ( ) .
,
.
( ).

CT : Hyperdense (Unenhanced) heterogeneous enhance
bony erosion (50%) but, hyperostosis rare
MRI : vascular flow voids,
heterogeneous enhancement
narrow based dural attachment
Angiography : corkscrew vascular configuration,
shunting, long-lasting vascular stain


Treatment

Aggressive surgery is the treatment of choice
Preoperative embolization .
adjuvant radiation .
Radiosurgery

Imaging
Recurrence

.

Metastasis
bone, lung, liver .
intra-axial seeding: very rare

Prognostic factor

Postoperative radiotherapy
.
More poor prog. than malignant MNG or atypical MNG
Periodic chest x-ray, liver function test. abnormal bone pain

Ch. 64
Meningeal Sarcoma
Chap.64 Meningeal Sarcoma
Clinical significance
Mesenchymal tissue.
dura, pia-arachnoid, stroma of choroids plexus, adventitial fibroblast,
tela choroidea
dural base parenchymal mass .

Pathology
Angiosarcoma
, Brain invasion
Chondrosarcoma
Dura, skull base
Rhabdomyosarcoma
.
myoglobin, myosin, muscle-specific actin, desmin immunohistochemistry

Pathology
Fibrosarcoma
intracranial sarcoma. RT
gliosarcoma or primary intracranial sarcoma
True fibrosarcoma sarcomatous degeneration of
meningioma
1. well known herringbone appearance
:fibrosarcoma
2. MNG EMA (+) fibrosarcoma EMA(-)
( EMA(-) MNG )
3. past history, whorls and psammoma body:
MNG


Primary meningeal sarcomatosis;
diffuse spread of sarcomatous tissue
. Subarachnoid space
spinal cord .

Ewings sarcoma

epidural space ( spine)
.
pituitary adenoma RT sarcoma (+)


Patient presentation, evaluation and
treatment

Bone erosion: MNG scalp erosion
sarcoma .
sarcoma - extra-axial lesion
sarcoma - intra-axial or intraventricular
lesion
MRI : Homogeneous enhancement
T2WI - hypointense signal (cellularity )

.

!