Hyperostosing Spheno-Orbital
Meningioma
Classification Paradigm
JOHN R. FLOYD, II M.D.
FRANCO DEMONTE, M.D.
UT, MD ANDERSON CANCER CENTER
UT, HEALTH SCIENCE CENTER SAN ANTONIO
Cushing, H. and L. Eisenhardt, Meningiomas. Their
classificaion, regional behavior, life history, and
surgical end results. 1938, Springfield, IL: Charles C.
Thomas.
en plaque meningiomas
 with carpet like growth patterns,
osseous invasion, and hyperostosis.
global meningiomas
 grow into the Sylvian fissure, and its
diameter is typically greater than the
base.
Incidence
 Castellano (1952) reviewed 608 cases of
meningiomas
111
(18.4%) were along the sphenoid ridge
15 (2.5%) were associated with hyperostosis
Incidence varies from 4-9% in case series
Castellano, F., B. Guidetti, and H. Olivecrona, Pterional meningiomas en
plaque. J Neurosurg, 1952. 9(2): p. 188-96
1950’s….conservative
 “It is true that vision on the affected side may be
severely impaired or entirely lost….Nevertheless….this
hardly seems to be sufficient reason to run the risk of a
10-15% mortality”
 “ The bone must be burred away, which is a rather
dangerous procedure, as the fraise (burr) might slip.
In one of our cases, this happened and the carotid
artery was torn completely off at its point of entrance
into the cranial chamber. This patient died two days
later from the effects of cerebral ischaemia.

Castellano, F., B. Guidetti, and H. Olivecrona, Pterional meningiomas en
plaque. J Neurosurg, 1952. 9(2): p. 188-96.
Bonnal, J., et al.,
Invading
meningiomas of the
sphenoid ridge. J
Neurosurg, 1980.
53(5): p. 587-99.
Group
Type
Location
Extension
A
en masse
Clinoid,
Upward or
Cavernous
Medial
Sinus, Medial
Sphenoid
wing
B
en plaque
Greater and Downward
Lesser
Sphenoid
Wings
C
en masse
Combines feature of each
Roser, F., et al.,
Sphenoid wing
meningiomas with
osseous involvement.
Surg Neurol, 2005.
64(1): p. 37-43;
discussion 43.
Group
I
II
III
IV
V
Location
Medial Sphenoid
Wing
Medial Sphenoid
Wing
Middle Sphenoid
Wing
Lateral Sphenoid
Wing
Extension
None
Cavernous
Sinus
None
None
En plaque
None
VI
En Plaque
Cavernous
Sinus
VII
Pure Intraosseous
None
Confusing Nomenclature
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
En masse
En plaque
Sphenoid ridge meningiomas
Pterional tumors en plaque
Hyperostosing lesions of the ala magna
Invading meningiomas of the sphenoid wing
Spheno-cavernous meningiomas
Intraosseous meningiomas
Osteomeningioma
Extradural meningiomas
Spheno-Orbital Meningiomas
Author
Yr
Patients
Mortality
Perm
Morbidity
Temp
Morbidity
Recurrence
1
Castellano, F
1952 15
3 (23%)
2
Columella, F
1974 3
0 (0)
0 (0)
0 (0)
NS
3
Bonnal, J
1980 21
4 (19%)
12 (41%)
---
3 (10%)
4
Dolenc, V
1979 10
2 (20%)
2 (20%)
5
Pompili, A
1982 49
2 (4 %)
1 (2%)
13 (27%)
6
McDermott, M
1990 8
0(0)
2 ( 25%)
8 (100%)
7
Gaillard, S
1995 21
1 (5%)
---
9 (43%)
3 (14%)
8
Carrizo, A
1998 48
2 (4%)
7 (15%)
11 (23%)
8 (17%)
9
De Jesus, O
2001 6
NS
NS
NS
2 (33%)
10
Honeybul, S
2001 15
0 (0)
4 (27%)
11 (73%)
2 (13%)
11
Roser, F1
2005 82
1 (1.4 %)
7 (9%)
11 (13%)
25 (30%)
12
Sandalcioglu, E 2005 16
0(0)
2 (13%)
12 (80%)
9 ( 60%)
13
Schick, U
2006 67
0 (0)
9 (13%)
14 (21%)
7 ( 10%)
14
Ringel, F
2007 63
2 (3.2%)
21 (33%)
23 (37%)
16 (25%)
15
Al_Mefty, O
2007 17
0 (0)
0 (0)
4 (24%)
Rationale
1. Multiple classification schemes.
2. Confusing nomenclature.
3. Currently, there are no Preoperative
Classification schemes
Purpose of the Preoperative Scale
 allows the surgeon to
carefully evaluate the
surgical condition,
 to determine risk factors
for and against a
procedure,
 to anticipate outcomes
and problems in the
postoperative period,
 to educate patients
Proposed Classification Scheme
1.
2.
3.
Osseous
invasion
Soft tissue
invasion
Presence of
cranial nerve
neuropathy
Osseous Invasion
Sphenoid Bone: Posterior Superior View
Osseous Score
Anterolateral greater sphenoid wing
Lesser sphenoid wing
Lateral orbital wall
Orbital roof
Lateral or superior orbital rim
Temporal squamousal bone
Temporal mandibular joint
Inferomedial greater sphenoid wing
•medial to basal foramina
Anterior clinoid process/optic canal
Body of sphenoid bone
1
2
3
Osseous Invasion
Anteriolateral and inferiomedial greater wing of
sphenoid affected; body of sphenoid, lesser
wing, and anterior clinoid are not involved.
Soft Tissue Invasion
Soft Tissue Score
Temporalis muscle / fossa
Infratemporal fossa
Temporal convexity dura
globoid intradural component
Intraorbital extraperiorbital
Periorbital membrane
Intraperiorbital extraconal
1
Dura of lateral cavernous sinus
Dura of the superior orbital fissure
Intraperiorbital intraconal extraapical
Optic canal
2
cavernous sinus
Intraperiorbital, intraconal, orbital
apex
Superior orbital fissure
3
Soft Tissue Invasion
Lateral temporal dura and small intradural component.
Soft Tissue Invasion
Lateral cavernous sinus, superior orbital fissure,
orbital apex
Cranial Nerve Neuropathy
Optic Neuropathy
None
Mild Optic Neuropathy
Relative afferent papillary defect
Decreased color vision
Acuity better or equal to 20/400
Mild optic nerve pallor
Visual field deficit
Moderate Optic Neuropathy
Acuity worse that 20/400
Optic nerve atrophy
Severe Optic Neuropathy
Light perception only
Cranial III, IV or VI palsy
Absent
Present
0
1
2
3
A
B
Proposed Scale for Hyperostosing
Spheno-Orbital Meningiomas (HSOMs)
Score
Grade
2-3
I
4-6
II
7-9
III
 Patients were identified from the
Demographics
Patients
Male
Female
Ages
Mean Age
Mean Follow-up
Previous Surgery
Perioperative Deaths
Deaths During
Follow-up
Recurrences
Mean time to
recurrence
20
4 (20%)
16 (80%)
31-83 (yrs)
54 (yrs)
41 (mos)
5 (25%)
0
2 (10%)
5 (25%)
32 (mos)
Departmental database from
February 1994 until April 2008.
 All meningiomas of the sphenoid
ridge, cavernous sinus, and orbit
were included if there was
associated hyperostosis.
Presenting Symptoms
Presenting Signs & Symptoms
N=20
%
Proptosis
18
90%
Vision loss
12
60%
Diplopia
9
45%
Temporal Swelling
4
20%
Aphasia
3
18%
CN Palsy
4
20%
Methods
 Patients had formal
ophthalmological evaluations pre
and postop.
 Patients had detailed neuroimaging including CT scan, MRI
brain =/- Gad with orbital fat sat
sequences.
 Bicoronal incision, extradural
approach was utilized, with
orbital and zygomatic osteotomies
as needed.
Preoperative Score
Preoperative Criteria and Accumulated Score
Patient
#
Bone
Score
Soft Tissue
Score
3
15
16
20
1
2
5
6
8
9
10
11
13
14
17
4
7
18
12
19
2
2
2
1
2
3
2
2
2
2
2
2
1
2
1
1
1
1
1
1
1
1
2
2
3
3
1
3
3
3
3
3
2
3
2
1
3
3
3
3
Cranial n.
III, IV, VI
Score
Total Score
Pre- operative
Grade
1
1
1
A
A
A
A
A
A
A
A
A
A
A
A
A
A
A
A
B
3
3
3
2
4
5
4
5
6
4
6
6
6
5
5
4
5
IA
IA
IA
IA
IIA
IIA
IIA
IIA
IIA
IIA
IIA
IIA
IIA
IIA
IIA
IIA
IIB
1
3
3
B
B
B
6
7
9
IIB
IIIB
IIIB
Optic
neuropathy
1
1
1
1
1
2
Outcomes Evaluated
1.
Clinical Outcomes
a. Proptosis
b. Vision
c. Diplopia
2.
Technical Outcomes
a. Extent of Resection ( Simpson Grade)
3.
Oncologic Outcome
a. Time to Progression
Clinical Outcomes: Proptosis
Symptom
Preop
Proptosis
1.
2.
3.
4.
5.
6.
7.
Postop
Improved
Worsened
New Morbidity
18
15 (83%)
0
0
233*
185 (79%)
0
5 (2%) (enopthalmus)
Roser, F., et al., Sphenoid wing meningiomas with osseous involvement. Surg Neurol, 2005. 64(1): p. 37-43;
discussion 43.
Shrivastava, R.K., et al., Sphenoorbital meningiomas: surgical limitations and lessons learned in their longterm management. J Neurosurg, 2005. 103(3): p. 491-7.
Bikmaz, K., R. Mrak, and O. Al-Mefty, Management of bone-invasive, hyperostotic sphenoid wing
meningiomas. J Neurosurg, 2007. 107(5): p. 905-12.
Honeybul, S., et al., Sphenoid wing meningioma en plaque: a clinical review. Acta Neurochir (Wien), 2001.
143(8): p. 749-57; discussion 758.
Ringel, F., C. Cedzich, and J. Schramm, Microsurgical technique and results of a series of 63 spheno-orbital
meningiomas. Neurosurgery, 2007. 60(4 Suppl 2): p. 214-21; discussion 221-2.
Sandalcioglu, I.E., et al., Spheno-orbital meningiomas: interdisciplinary surgical approach, resectability and
long-term results. J Craniomaxillofac Surg, 2005. 33(4): p. 260-6.
Schick, U., et al., Management of meningiomas en plaque of the sphenoid wing. J Neurosurg, 2006. 104(2): p.
208-14.
Proptosis
 No statistical trend across preoperative grades, eg,
Grade IA vs Grade IIIB.
 The bone score as an independent variable did not
predict the Simpson Grade of resection or
recurrence/progression.
Clinical Outcomes: Vision
Symptom
Preop
Vision
Improved
Worsened
New Morbidity
12
4 (42%)
0
0
133*
58 (43%)
3 (2%)
1 (< 1%)
Optic Neuropathy
Preoperative
Postop
Postoperative Improvement
# Patients
APD
12
CD
6
ECF
4
*< or = 20/400 4
LPO
2
APD
0
CD
ECF
*< or +
20/400
LP
O
1
2
3
0
APD =Afferent Pupillary Defect; CD = Color Desaturation; ECF = Enlarged Central Field; * Acuity; LPO = Light
Perception Only
Clinical Outcomes: Vision
 Positive trend toward absence of Optic Neuropathy (ON)
and Simpson Grade I resection and Preoperative Grade IA.
(not statistically significant).
 Positive trend (p=0.074) toward the presence of ON and Simpson
Grade IV resection (not statistically significant).
 Presence of Optice Neuropathy as an independent variable
did not predict the Simpson Grade of resection or
progression or recurrence
Clinical Outcomes: Diplopia
 4/9 (44%) patients diplopia resolved
 Double vision caused from rectus muscle constriction.
 Orbital decompression relieved symptoms.
• 5/9 (55%) patients had unchanged diplopia
 4 were due to true CN III, IV or VI palsy
 1 pt had previous TBI
• 1 patient developed delayed double vision due to lateral
rectus fibosis
Diplopia
• Positive trend for the absence of CN palsy in the
preoperative Grade IA HSOMs (not statistically significant).
• The presence of cranial nerve palsy did not
independently predict the Simpson Grade of
resection, progression or recurrence.
Clinical Outcomes
Clinical Outcome: Improved (I); Stable (S), Worse
(W)
Summary of Clinical Outcomes
Grade
IA
IIA
IIB
IIIB
Patient #
Proptosis
3
15
16
20
1
2
5
6
8
9
10
11
13
14
17
4
7
18
12
19
I
I
I
I
ON
Diplopia
I
I
I
I
I
I
I
I
I
I
I
S
I
I
S
S
S
I
S
I
S
I
I
S
I
S
S
S
S
I
S
W
S
S
S
Technical and Oncological Outcomes
Preoperative Grade
HSOM
IA
IIA
IIB
IIIB
Technical Outcome
Oncologic Outcome
Patient #
Simpson Grade
(extent of resection)
Time to Progression
(Months)
3
15
16
20
1
2
5
6
8
9
10
11
13
14
17
4
7
18
12
19
I
I
I
I
I
I
I
IV
IV
IV
IV
IV
IV
IV
IV
I
IV
IV
II
IV
9
96
24
18
12
12
Technical and Oncological Outcomes
Series
Yr
Followup
average
(mos)
# Pts
Recurrenc Time to
Average
e (%)
Recurrence Time to
(mos)
Recurrence
(mos)
Honeybul, S
2001
40
15
2 (13%)
36,96
66
Roser, F
2005
66
82
25 (30%)
Not specified
32
Sandalcioglu, E
2005
68
16
9 ( 60%)
16,118,13,66,4
7,62,9,12, 17
40
Shrivastava, R
2005
60
25
2 (8%)
12, 132
73
Schick, U
2006
46
67
7 ( 10%)
29,21,47,21,14,
23,13
24
Ringel, F
2007
54
63
16 (25%)
Not Specified
Not Specified
Bikmaz, K.
2007
36
17
1 (6%)
72
72
DeMonte
2009
42
20
5 (25%)
96,24,18,12,9
32
Technical and Oncological Outcomes
 Positive trend toward HSOM Grade IA and Simpson Grade I with no
recurrences (not statistically significant).
 The individual bone score, presence or degree of optic neuropathy,
presence or absence of cranial nerve palsy did not predict Simpson Grade
resection, progression, or recurrence
 Soft tissue score was highly predictive of Simpson Grade resection ( p<0.001)
 When grouping soft tissue score 1 +2 vs. 3, this did predict tumor
progression. ( p <0.045)
 When analyzed as a continuous variable,

the hazard score for the total score ( eg 2-9; not by Grade), is 3.7, 95% CI
0.95 – 14.4, with a p value close to significance p = 0.06
Oncologic Outcomes
 Areas of progression:
 cavernous sinus (two),
 orbital apex and cavernous sinus (two),
 and intraorbital (one).
 All patients have had tumor stabilization with either:
 SRS
 IMRT
Other Variables
No statistical difference between preoperative
score or grade and:
 Gender
 Histological
 MIB
grade
rate
Progression was unrelated to MIB rate or histology
Positive trend between higher preoperative grade
and increasing age
(not statistically significant).
 Overall, proptosis will improve 80%
Conclusions
Limitations
Limited
number of
patients
Short followup for certain
patients
of time
 Vision will improve about 40% of time
( no improvement if LPO)
 Cranial nerve III,IV, or VI palsy tend
not to improve
 The strongest correlation with
predicting outcomes is the soft tissue
score:



Extent of resection ( Simpson Grade)
Risk for Progression
Progression occurs from residual tumor in
the cavernous sinus, superior orbital fissure,
orbital apex
 Trends
Conclusions



Grade IA:
 Improved Clinical Symptoms
 Complete Resections
 No recurrences
Grade IIA:
 Improved or Stabilized Clinical
Symptoms
 Incomplete Resections
 More likely to have
recurrence/progression
Grade IIB or IIIB
 Stabilized Clinical Symptoms
 Incomplete Resections
 More likely to have
recurrence/progression
Summary
 The approach to HSOMs has
shifted
from a nonsurgical stance,
 to a present day patient outcome
oriented strategy.
