JOHN R. FLOYD, II M.D.FRANCO DEMONTE, M.D.

U T, M D A N D E R SO N C A N C E R C E N T E R U T, H E A LT H S C I E N C E C E N T E R SA N A N T O N I O

Hyperostosing Spheno-Orbital Meningioma Classification Paradigm

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.

Cushing, H. and L. Eisenhardt, Meningiomas. Their classificaion, regional behavior, life history, and surgical end results. 1938, Springfield, IL:

Charles C. Thomas.

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.

Group Type Location Extension

A en masse Clinoid, Cavernous

Sinus, Medial

Sphenoid wing

Upward or

Medial

B en plaque Greater and Lesser Sphenoid

Wings

Downward

C en masse Combines feature of each

Bonnal, J., et al., Invading meningiomas of the sphenoid ridge. J Neurosurg, 1980. 53(5): p. 587-99.

Roser, F., et al., Sphenoid wing meningiomas with osseous involvement. Surg Neurol, 2005. 64(1): p. 37-43; discussion 43.

Group

Location Extension

IMedial Sphenoid

WingNone

IIMedial Sphenoid

WingCavernous

Sinus

IIIMiddle Sphenoid

WingNone

IVLateral Sphenoid

WingNone

V En plaque None

VI En PlaqueCavernous

Sinus

VIIPure

IntraosseousNone

Confusing Nomenclature

1. En masse2. En plaque3. Sphenoid ridge meningiomas4. Pterional tumors en plaque5. Hyperostosing lesions of the ala magna6. Invading meningiomas of the sphenoid wing7. Spheno-cavernous meningiomas8. Intraosseous meningiomas9. Osteomeningioma10. Extradural meningiomas11. 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%)

1. Multiple classification schemes.

2. Confusing nomenclature.

3. Currently, there are no Preoperative Classification schemes

Rationale

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. Osseous invasion

2. Soft tissue invasion

3. Presence of cranial nerve neuropathy

Osseous InvasionSphenoid Bone: Posterior Superior View

Osseous Score Anterolateral greater sphenoid wingLesser sphenoid wingLateral orbital wall Orbital roof Lateral or superior orbital rimTemporal squamousal bone Temporal mandibular joint

1

Inferomedial greater sphenoid wing• medial to basal foramina

Anterior clinoid process/optic canal2

Body of sphenoid bone 3

Anteriolateral and inferiomedial greater wing of sphenoid affected; body of sphenoid, lesser wing, and anterior clinoid are not involved.

Osseous Invasion

Soft Tissue Invasion

Soft Tissue ScoreTemporalis muscle / fossaInfratemporal fossaTemporal convexity duragloboid intradural component Intraorbital extraperiorbitalPeriorbital membraneIntraperiorbital extraconal

1

Dura of lateral cavernous sinusDura of the superior orbital fissureIntraperiorbital intraconal extra-apicalOptic canal

2

cavernous sinusIntraperiorbital, intraconal, orbital apexSuperior 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 NeuropathyNone 0Mild Optic Neuropathy Relative afferent papillary defect Decreased color vision Acuity better or equal to 20/400 Mild optic nerve pallor Visual field deficit

1

Moderate Optic Neuropathy Acuity worse that 20/400 Optic nerve atrophy

2Severe Optic Neuropathy Light perception only 3Cranial III, IV or VI palsyAbsent APresent B

Score Grade

2-3 I

4-6 II

7-9 III

Proposed Scale for Hyperostosing Spheno-Orbital Meningiomas (HSOMs)

Patients were identified from the 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.

Patients 20

Male 4 (20%)

Female 16 (80%)

Ages 31-83 (yrs)

Mean Age 54 (yrs)

Mean Follow-up 41 (mos)

Previous Surgery 5 (25%)

Perioperative Deaths 0Deaths During Follow-up 2 (10%)

Recurrences 5 (25%)Mean time to recurrence 32 (mos)

Demographics

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%

Presenting Symptoms

MethodsPatients had formal

ophthalmological evaluations pre and postop.

Patients had detailed neuro-imaging 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

Optic neuropathy

Cranial n. III, IV, VI

ScoreTotal Score

Pre- operative Grade

3 2 1   A 3 IA15 2 1   A 3 IA16 2 1   A 3 IA20 1 1 A 2 IA1 2 1 1 A 4 IIA2 3 2   A 5 IIA5 2 2   A 4 IIA6 2 3   A 5 IIA8 2 3 1 A 6 IIA9 2 1 1 A 4 IIA

10 2 3 1 A 6 IIA11 2 3 1 A 6 IIA13 1 3 2 A 6 IIA14 2 3   A 5 IIA17 1 3 1 A 5 IIA4 1 2 1 A 4 IIA7 1 3 1 B 5 IIB

18 2 3 1 B 6 IIB12 1 3 3 B 7 IIIB19 3 3 3 B 9 IIIB

Outcomes Evaluated

1. Clinical Outcomesa. Proptosisb. Visionc. Diplopia

2. Technical Outcomesa. Extent of Resection ( Simpson Grade)

3. Oncologic Outcomea. Time to Progression

Clinical Outcomes: Proptosis

1. Roser, F., et al., Sphenoid wing meningiomas with osseous involvement. Surg Neurol, 2005. 64(1): p. 37-43; discussion 43.

2. Shrivastava, R.K., et al., Sphenoorbital meningiomas: surgical limitations and lessons learned in their long-term management. J Neurosurg, 2005. 103(3): p. 491-7.

3. Bikmaz, K., R. Mrak, and O. Al-Mefty, Management of bone-invasive, hyperostotic sphenoid wing meningiomas. J Neurosurg, 2007. 107(5): p. 905-12.

4. Honeybul, S., et al., Sphenoid wing meningioma en plaque: a clinical review. Acta Neurochir (Wien), 2001. 143(8): p. 749-57; discussion 758.

5. 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.

6. 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.

7. Schick, U., et al., Management of meningiomas en plaque of the sphenoid wing. J Neurosurg, 2006. 104(2): p. 208-14.

Symptom Preop Postop

Proptosis Improved Worsened New Morbidity

18 15 (83%) 0 0

233* 185 (79%) 0 5 (2%)

(enopthalmus)

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

Optic Neuropathy Postoperative Improvement    

Preoperative

# Patients APD CD ECF*< or + 20/400

LPO

APD 12 0  CD 6   1  ECF 4   2   *< or = 20/400 4   3  LPO 2   0

             

Symptom Preop Postop Vision

Improved Worsened New Morbidity12 4 (42%) 0 0

133* 58 (43%) 3 (2%) 1 (< 1%)

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

Summary of Clinical OutcomesClinical Outcome: Improved (I); Stable (S), Worse (W)

Grade Patient # Proptosis ON Diplopia

IA

3 I  15 I  16 I I20 I  

IIA

1   I  2 I I5  6 I I8 I S  9 I I  

10 I S  11 I I  13 I I I14 I  17 S S S4 I I W

IIB 7 I S S18 S S  

IIIB 12 S S S19 S S S

Technical and Oncological Outcomes

Technical OutcomeOncologic Outcome

Preoperative Grade HSOM

Patient # Simpson Grade

(extent of resection)

Time to Progression

(Months)

IA

3 I  15 I  16 I  20 I  

IIA

1 I  2 I  5 I  6 IV 98 IV 969 IV  10 IV 2411 IV 1813 IV 1214 IV  17 IV  4 I  

IIB 7 IV  18 IV  

IIIB 12 II 1219 IV  

Technical and Oncological Outcomes

Series Yr Followup average (mos)

# Pts Recurrence (%)

Time to Recurrence (mos)

Average Time to 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%) 16,118,13,66,47,62,9,12, 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 grade MIB rate

Progression was unrelated to MIB rate or histology

Positive trend between higher preoperative grade and increasing age (not statistically significant).

Conclusions

Overall, proptosis will improve 80% 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

Limitations Limited

number of patients

Short follow-up for certain patients

Conclusions

Trends 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

SummaryThe approach to HSOMs has

shifted

from a nonsurgical stance, to a present day patient

outcome oriented strategy.