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Neurosurg Focus 10 (5):Article 5, 2001, Click here to return to Table of Contents

Meningiomas of the orbit: contemporary considerations


PAUL T. BOULOS, M.D., AARON S. DUMONT, M.D., JAMES W. MANDELL, M.D., PH.D., AND
JOHN A. JANE, SR., M.D., PH.D.
Departments of Neurological Surgery and Pathology, University of Virginia Health Sciences Center,
Charlottesville, Virginia
Meningiomas are the most frequently occurring benign intracranial neoplasms. Compared with other intracranial
neoplasms they grow slowly, and they are potentially amenable to a complete surgical cure. They cause neurological
compromise by direct compression of adjacent neural structures. Orbital meningiomas are interesting because of their
location. They can compress the optic nerve, the intraorbital contents, the contents of the superior orbital fissure, the
cavernous sinus, and frontal and temporal lobes. Because of its proximity to eloquent neurological structures, this
lesion often poses a formidable operative challenge. Recent advances in techniques such as preoperative embolization
and new modifications to surgical approaches allow surgeons to achieve their surgery-related goals and ultimately optimum patient outcome. Preoperative embolization may be effective in reducing intraoperative blood loss and in improving intraoperative visualization of the tumor by reducing the amount of blood obscuring the field and allowing unhurried microdissection. Advances in surgical techniques allow the surgeon to gain unfettered exposure of the tumor while
minimizing the manipulation of neural structures. Recent advances in technologynamely, frameless computer-assisted image guidanceassist the surgeon in the safe resection of these tumors. Image guidance is particularly useful
when resecting the osseous portion of the tumor because the tissue does not shift with respect to the calibration frame.
The authors discuss their experience and review the contemporary literature concerning meningiomas of the orbit and
the care of patients harboring such lesions.

KEY WORDS meningioma orbital tumor surgical resection

There is nothing in the whole realm of surgery more


gratifying than the successful removal of a meningioma
with subsequent perfect functional recovery, especially
should a correct pathological diagnosis have been previously made. The difficulties are admittedly great, sometimes insurmountable, and though the disappointments
still are many, another generation of neurological surgeons will unquestionably see them largely overcome.
Harvey Cushing,26 1922.
Meningiomas are the most common benign intracranial
lesions accounting for approximately 18% of all intracranial neoplasms.98 Meningiomas have kindled much interest over the years because of their biological and clinical
behavior and of the possibility of cure with appropriate
therapy. Virchcow125 was the first to describe the classic
pathological feature of the meningiomanamely, the
psammoma body (sandlike, referring to the presence of
granules within the tumor). The nomenclature of these tumors evolved with time; numerous descriptions and considerable ambiguity have been generated by scholars. To
eliminate such confusion Cushing26 in 1922 introduced
the simple yet all-encompassing term meningioma. Subsequently, meningiomas as a pathological entity have
Abbreviations used in this paper: CT = computerized tomography; MR = magnetic resonance.

Neurosurg. Focus / Volume 10 / May, 2001

assumed an important role in the evolution of the specialty of neurological surgery. Some individuals have even
asserted that meningiomas have had the most significant
impact in the development of our specialty.71,72 MacCarty71 wrote, If we were to designate an intracranial
neoplasm that has had the most effect on the development
of neurologic surgery, very likely the intracranial meningioma would be prominently considered.
Meningiomas with orbital involvement have captured
the interests of neurosurgeons, neurologists, and ophthalmologists alike for over a century. Members from each of
these specialties have contributed to the treatment of these
lesions. In the not-so-distant past, however, a collegial
relationship did not always exist among practitioners. In
1938 Cushing reminisced, [In] recent years, the ophthalmic surgeon, the earliest surgical specialist, and the neurosurgeon, the latest, have from the opposite directions
come to meet at the barrier of the optic forameneach
somewhat hesitant to trespass on the others field of
work.27 The successful treatment of these fascinating yet
often formidable lesions has required the harmonious
amalgamation of the expertise of ophthalmological and
neurosurgical surgeons. Indeed, comprehensive and multidisciplinary cranial base teams have been assembled at
many institutions to treat patients harboring orbital meningiomas and other lesions of the cranial base.
1

P. T. Boulos, et al.
Meningiomas of the orbit can be thought of as primary
and secondary in origin. Primary orbital meningiomas
arise from the optic nerve sheath and may extend through
the optic canal intracranially, or they may infiltrate the
sphenoid wing producing hyperostosis. Nearly two thirds
of orbital tumors, however, originate from outside the
confines of the orbit.10,22,49,60,126 Secondary orbital meningiomas usually arise from the inner and outer aspects of
the sphenoid wing but not the middle sphenoid wing.
They may infiltrate medially into the orbit, often with
intraorbital, intracranial, and intraosseous portions. They
often compress the optic nerve by narrowing the optic
canal. They may infiltrate the contents of the supraorbital
fissure and cavernous sinus or compress the frontal and
temporal lobes. There may also be cases of primary intraosseous meningiomas in which the tumor arises from
within the bone. Tumors originating along the sphenoid
wing have a relatively high incidence of bone involvement.95 It is thought that nearly 30% of orbital meningiomas may involve bone (thus producing so-called hyperostosis), and 12% may originate from within the orbital
bones.102
The primary purpose of the present discussion is to address comprehensively the unique topic of meningiomas
of the orbit. Data from the authors experience will be
included and the contemporary literature will be reviewed.
Additionally, a recent case, complete with radiographic
and pathological data and intraoperative video clips will
be used to illustrate many of the salient points germane to
meningiomas of this region.
EPIDEMIOLOGY
Meningiomas comprise approximately 18 to 20% of all
intracranial tumors90 and are the most common benign intracranial neoplasm and the second most common intracranial tumor overall (second to only gliomas). Intracranial meningiomas have been estimated to occur with an
incidence of 2.1/100,000 people.101
Meningiomas account for 3 to 9% of all orbital tumors49,99 and are certainly not considered rare.81 Primary
orbital meningiomas represent between 0.4 to 2% of all
intracranial meningiomas.19,72,101 Secondary orbital meningiomas are considerably more common than their primary counterparts. Sphenoid ridge meningiomas comprise
between 16 to 20% of all meningiomas90,96,130 whereas meningiomas arising from the suprasellar region account for
7 to 30% of intracranial meningiomas.90,130
Orbital meningiomas, as with those located in other regions, have a predilection for afflicting females (73 to
84% occur in females19,131), although this female preponderance does not appear to hold true in children with orbital meningiomas30 and in meningiomas in general in
the pediatric population.34 Demographic data concerning
the age distribution of meningiomas has been the subject of some controversy. Although reported frequently in
younger patients, particularly in the early literature,8,11,12,
28,33,52,79,80,122
others have found these tumors to be relatively uncommon in patients younger than 20 years of
age.53,58,68 Reports of orbital meningiomas in children may
have been erroneous in their diagnosis, as gliomas can
evoke a surrounding dural reaction that superficially mim2

ics meningioma.21,37 Suffice it to say that meningiomas are


overall uncommon in children (1.5 to 2.3% of all intracranial tumors24); however, the relative incidence of orbital
involvement in childhood meningiomas has increased.
Several groups have endeavored to determine the predisposing factors for orbital meningiomas. Neurofibromatosis appears to be one predisposing factor, particularly in
pediatric meningiomas.47,58,67,83,88,106,108,120,126 Exposure to
radiation has clearly been associated with meningioma
formation (with a latency ranging from 1227 years),
although there have been few cases in which orbital involvement is described. The role of trauma and viral
infection in the development of meningiomas is controversial.
CLINICAL PRESENTATION
The clinical presentation of orbital meningiomas has
not changed since it was described by Cushing and
Eisenhardt27 in 1938. Unilateral visual loss and progressive, usually painless exophthalmos are the most common presentations described in the literature.21,97,105,124,131
Visual acuity tends to be involved early and exophthalmos
manifests later.131 The apparent visual loss generally develops gradually, although acute visual loss may occur in
2 to 5% of intracanalicular and 8 to 12% of intracranial
meningiomas.128,129 Other relatively common clinical features include optic disc changes, diplopia, headaches, and
nausea and vomiting. Optic nerve changes may be due to
intracranial hypertension, which causes papilledema, or
direct compression on the optic nerve, resulting in papilledema or optic atrophy. Additionally, intracranial hypertension may produce papilledema in the contralateral eye.
Diplopia may be caused by cranial neuropathies or by
direct disruption of the rectus muscles. Headache, as well
as nausea and vomiting, are typically associated with intracranial hypertension. Wright131 emphasized that visual
loss was the most common initial symptom, followed by
exophthalmos, diplopia, afferent papillary defect, and optic nerve changes with shunt vessels present. So-called optociliary shunt vessels on the disc surface, representative
of collateral circulation between the central retinal vein
and the choroidal/ciliary venous drainage system,87 are
strongly suggestive of meningiomas involving the anterior visual pathways.
PATHOLOGICAL CHARACTERISTICS
The pathological origin of meningiomas is thought to
be the arachnoid cap cells. The common sites for the tumor coincide with the presence of arachnoid villi, and
they are often tightly bound to areas of arachnoid granulation tissue.4 Hence, the majority of meningiomas originate
in parasagittal, cavernous sinus, tuberculum sellae, sphenoid wing, lamina cribosa, foramen magnum, and torcular
regions.18 Cushing must have appreciated this characteristic distribution, as he described meningiomas by their
common locations and clinical syndromes.26
Meningiomas of the orbit have similar pathological
characteristics to other intracranial meningiomas. They
exhibit various histological subtypes. Cushing and Eisenhardt27 described nine main types and 20 subtypes. In the
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Orbital meningiomas
revised World Health Organization classification 15 subtypes are identified (Table 1). Meningothelial meningiomas are solid lobulated masses or sheets of meningothelial
cells, in which the cell membrane is not well defined, and
this gives an overall appearance of a syncytium; mitotic
activity is low. Fibrous meningiomas are composed of
bundles of cells that resemble fibroblasts; they typically
contain collagen and reticulin, and whorl formation and
psammoma bodies may be focally present. Transitional
meningiomas are a mixture of the meningothelial and fibrous types.65 Psammomatous meningiomas contain many
psammoma bodies. Bone-related proteins, including osteopontin, are produced by CD68-positive macrophages
and may play a role in calcified psammoma body formation.50 Angiomatous meningiomas contain blood vessels
of different shapes and sizes, typically with an endothelial
lining. Microcystic meningiomas contain both macroscopic and intracellular microscopic cysts filled with eosinophilic mucin; microcyst formation may be due to
tumor cell secretions, tumor cell degeneration, cerebrospinal fluid, penetration into the tumor, or vascular
changes. Secretory meningiomas are typically meningiothelial or transitional lesions in which epithelial differentiation has produced glandular structures. Clear cell meningiomas contain sheets of polygonal cells with clear
cytoplasm. The cytoplasm stains positive for periodic acidSchiff because of the presence of glycogen. Chordoid
meningiomas are composed of eosinophilic, vacuolated
cells in trabeculae, resembling chordomas. Lymphoplasmacyte-rich meningiomas are typically meningothelial,
fibrous, or transitional with a lymphocytic infiltrate. Metaplastic meningiomas are meningothelial, fibrous, or

TABLE 1
World Health Organization classification of meningiomas*
Type of Meningioma

benign
meningothelial
fibrous (fibroblastic)
transitional (mixed)
psammomatous
angiomatous
microcystic
secretory
lymphoplasmacyte-rich
metaplastic
aggressive
atypical
clear cell (intracranial)
chordoid
rhabdoid
papillary
anaplastic

WHO Grade

I
I
I
I
I
I
I
I
I
II
II
II
III
III
III

* Adapted from Louis DN, Scheithauer BW, Budka H, et al: Meningiomas, in Kleihues P, Cavenee WK (eds): Pathology and Genetics of Tumours of the Nervous System. Lyon: IARC Press, 2000, 176184.
Abbreviation: WHO = World Health Organization.
Defined as those with a low risk of recurrence and aggressive growth
behavior.
Defined as those with a greater risk of recurrence and aggressive
growth behavior.

Neurosurg. Focus / Volume 10 / May, 2001

transitional with metaplastic changes that may be cartilaginous, osseous, xanthomatous, myxoid, or lipomatous.65
Atypical meningiomas deviate to some extent from the
aforementioned benign features. They contain increased
mitotic activity, increased cellularity, small cells, prominent nucleoli, sheet-like growth, and areas of necrosis.
Typically they are associated with a greater rate of recurrence.16,65 Rhabdoid meningiomas are uncommon. They
are aggressive tumors that typically consist of patches or
sheets of rhabdoid cells. Rhabdoid cells are round with
eccentric nuclei and prominent nucleoli. The cells also
contain an eosinophilic cytoplasm with whorls of intermediate filaments.69 Papillary meningiomas are composed
of meningothelial cells in a radiating pattern resembling
pseudorosettes. They have a propensity for metastasis and
recurrence. Anaplastic meningiomas have a more malignant histological pattern than the atypical meningiomas.
They contain higher mitotic activity and greater necrosis.
Their incidence is fortunately relatively low, ranging from
0.9 to 10.6% in different series, with an overall mean representation of 2.8% of meningiomas.104
Primary orbital meningiomas arising from the optic
sheath typically consist of the transitional histological
subtype.2 In addition, psammomatous changes have been
noted in orbital meningiomas.23 Other histological subtypes including fibrous, papillary, and anaplastic meningiomas typically do not involve the orbit primarily.103 In
our evolving understanding of the development of optic
sheath meningiomas we have witnessed the replacement
of one hypothesisthat they arise primarily extradurally
with secondary invasion of the optic dural sheath123with
anotherthat they arise from the dural sheath itself.22
These lesions then spread along the optic nerve, often enveloping the nerve completely and thereby encompassing
the pial blood supply.5 The optic nerve may be compromised by direct invasion by tumor or by compression
leading to atrophy.96,107 Furthermore, tumor extension beyond the dura may affect the extraocular muscles.110
As previously mentioned, meningiomas with orbital involvement, particularly those originating from the sphenoid wing, commonly demonstrate involvement of the
bone. Osseous involvement may consist of hyperostosis
associated with an extraosseous meningioma, or the tumor
may originate within the bone itself.
Hyperostosis associated with intracranial meningiomas
is a well-recognized phenomenon. The reported incidence
varies from 252527 to 49%.40,91,111 Numerous theories have
emerged to account for hyperostosis and include vascular
disturbances of the bone induced by the tumor,13,41,48,64,86,92
preceding trauma,3,7,9,27,42,92,112,113,115,117 osseous reaction to
the adjacent tumor without tumor invasion,25,59,93 production of bone by the tumor itself,41,48,64,86,92 stimulation of
osteoblasts in adjacent normal bone by tumor-secreted
factors,36,44,78,95 and tumor invasion of the bone.13,15,29,36,
44,61,78,95
Recent and commendable work reported by AlMeftys group lends credence to the hypothesis that hyperostosis results from tumor invasion of the bone.94
These authors demonstrated this finding through careful
radiographic and surgical/histopathological correlation of
meningiomas involving the cranial base. The origin of
hyperostosis is important in the surgical management of
meningiomas. Given that hyperostosis is likely secondary
3

P. T. Boulos, et al.
to tumor invasion and that complete tumor removal is the
desired goal, resection of the hyperostotic bone becomes
mandatory.
RADIOLOGICAL FINDINGS
Optic nerve sheath meningiomas may appear normal on
plain x-ray films in the early stages of tumor development.
Late in tumor development, optic canal enlargement or
hyperostosis of the optic canal may be observed. Computerized tomography scanning of specifically optic nerve
sheath meningiomas may reveal thickening of the optic
nerve or the tram track sign in which two strips of lucency are seen around the center of the enlarged optic
nerve, signifying that the optic nerve is surrounded by
tumor.74 This feature is in contrast to optic nerve gliomas
in which the optic nerve itself is expanded.105 The presence of calcification is an important feature that is highly
suggestive of meningioma. Hyperostosis of the optic canal
may also be evident on CT scans. Contrast administration
typically reveals homogeneous enhancement of the mass.
Magnetic resonance imaging can provide more details
about the tumor; specifically it can help differentiate fusiform lesions and loculated from eccentric lesions. Additionally MR imaging can also detect smaller lesions. En
plaque lesions can be seen on gadolinium-enhanced fatsuppressed T1-weighted MR images as two strips of enhancement on either side of the optic nerve.74
Either hyperostosis or erosion of the orbital bone may
be seen on radiographs of Sphenoid ridge meningiomas.
Computerized tomography examination with and without
contrast medium can often reveal the extent of the tumors
intracranial involvement into the anterior and middle cranial fossae. It can also further delineate the involvement of
the bone with hyperostosis or tumor infiltration. Additionally, CT scanning can demonstrate the extent of intraorbital involvement. Magnetic resonance imaging can
provide finer detail of the anatomical location of the
tumor. A clear dural tail, which would support the diagnosis of meningioma, may also be visualized using MR
imaging.
DIFFERENTIAL DIAGNOSIS
An understanding of orbital anatomy facilitates the formulation of a differential diagnosis for orbital meningiomas. Lesions may arise from the optic nerve and its
adjacent structures (Table 2). A conglomeration of pathological entities may affect the optic nerve and surrounding orbital contents. Enlargement of the optic nerve may,
for example, be due to tumor involvement in addition to a
multitude of inflammatory conditions or vascular conditions.87 Masses arising from the surrounding vicinity may
also result from a host of conditions.
NATURAL HISTORY
Rational treatment decisions regarding the treatment of
orbital meningiomas are predicated on an appreciation of
the natural history of the disease as well as a detailed
understanding of the available treatment modalities and
their associated benefits, limitations, and risks. It was
4

TABLE 2
Differential diagnosis for orbital lesions
apparent originoptic nerve
tumors
meningioma (from the optic nerve sheath)
glioma (benign or malignant)
neuroma/neurofibroma (plexiform neuroma w/ neurofibromatosis)
metastatic (lung/breast/prostate cancer, leukemic infiltration,
neuroblastoma, Ewing tumor
other (arachnoid cysts, juvenile xanthogranulomas)
inflammatory conditions
multiple sclerosis optic neuritis
infectious optic neuritis (syphilis, viral)
other (sarcoidosis)
vascular conditions
central retinal vein occlusion
apparent originadjacent regions
meningioma (sphenoid wing, cavernous sinus, suprasellar)
glioma
cavernous hemangioma
hemangioblastoma
hemangiopericytoma
fibrous dysplasia
bone tumor
infectious process (abscess)
aneurysm
malignant sinonasal tumor (squamous cell carcinoma, rhabdomyosarcoma)
metastasis
dermoid tumor
benign mixed lacrimal gland/mucocele
cholesteatoma
venous varix
arteriovenous malformation
nonspecific orbital inflammation

originally thought, based on anecdotal reports, that meningiomas affecting the orbit did not portend a clinical course
of progression and neurological deterioration.6,38,114 As experience has accrued and long-term follow-up data have
become available, however, it has become apparent that
these tumors almost invariably progress43,51,70,105,106,121 and
may grow to overwhelming size with time.55
As mentioned previously, orbital meningiomas afflicting pediatric patients may be associated with a poorer
prognosis than in older patients.2,84,126 Analysis of some
data suggests that in the pediatric population, these tumors
may not only produce visual impairment but also may
extend to adjacent areas such as the cavernous sinus, sellar region, the anulus of Zinn, and the pterygomaxillary
fossa,13 particularly with tumor involvement of the medial sphenoid wing.14 With tumor extension, complete extirpation without significant neurological compromise
becomes exceedingly difficult.32
Thus it appears that meningiomas with orbital involvement progress, leading to potential neurological compromise. This is particularly so in the younger population in
whom these tumors appear to behave more aggressively.
ILLUSTRATIVE CASE
History and Examination. This 35-year-old right-handed woman presented to an ophthalmologist with a history
of right eye swelling and exophthalmos. She complained
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Orbital meningiomas

Fig. 1. Left: Axial gadolinium-enhanced T1-weighted MR image revealing an enhancing lesion at the tip of the right
temporal lobe, widening of the lateral wall of the orbit, compression of the orbit, and exophthalmos. This image demonstrates the classic characteristics of a sphenoid wing meningioma: intracranial invasion, intraorbital compression, and
sphenoid wing expansion. Center: Coronal gadolinium-enhanced T1-weighted MR image revealing widening of the lateral wall of the orbit, orbital compression, and enhancement of the subfrontal dura. Widening of the lateral wall of the
orbit and intraorbital compression can be observed. Right: External carotid artery angiogram demonstrating several
feeding vessels off of the middle meningeal artery feeding the sphenoid wing mass. The classic feature of meningiomas,
tumor blush, can also be observed.

of occasional blurry vision and intermittent headaches. On


physical examination right-sided exophthalmos was observed, yet otherwise she was neurologically intact. Findings on cranial nerve and sensorimotor examinations were
within normal limits.
Peroperative Treatment. The patient underwent MR
imaging and CT examination. As seen in Fig. 1 left and
center, these studies revealed an enhancing lesion anterior
to the tip of the right temporal lobe, as well as widening of
the sphenoid ridge with extension of the intraosseous
mass into the orbit. Endovascular embolization was performed. As seen in Fig. 1 right, external carotid artery angiography revealed a tumor blush, as is frequently seen in
meningiomas. The tumor was embolized through selective catheterization of the middle meningial artery. There
was no angiographic evidence of the tumors blood supply
being derived from the internal carotid artery system.
Operation. The patient was taken to the operating room
the day after endovascular embolization. A frontotemporal craniotomy was performed. The tumor portion extending into the sphenoid ridge was resected in an extradural
fashion. As seen in Video Clip 1, the lateral wall of the
orbit was removed to the anterior clinoid process. The soft
intraorbital portion of the tumor was then removed.
VIDEO CLIP 1. Osseous bone removal. This is an intraoperative
video edited to depict several key points of the osseous removal. A
variety of clips demonstrates drilling of the sphenoid ridge. The
drill is used to thin the tumor-infiltrated lateral wall of the orbit.
The punch is then used to remove the thinned wall to the orbit. The
final frames demonstrate the absence of the greater and lesser sphenoid ridges, as well as the contents of the supraorbital fissure left
intact.

As demonstrated in Video Clip 2, the intradural portion


of the tumor was then removed. The procedure was performed with the assistance of computer image guidance,
which was found to be particularly useful in this case
because much of the tumor was intraosseous. The frameNeurosurgical Focus / Volume 10 / May, 2001

less guidance system is calibrated to the skull with a rigid


fixation system. Over the course of the operation the
osseous structures do not shift with retraction or decompression; thus, the skull base was believed to be accurate
(Fig. 2).
VIDEO CLIP 2. Soft tumor removal. This is an intraoperative
video demonstrating the removal of the intracranial soft tumor,
which was performed in a standard fashion by using bipolar electrocautery and microinstruments.

Postoperative Course. The patient awoke from anesthesia without cranial nerve dysfunction. After postoperative
swelling had subsided, the exophthalmos resolved. Final
pathological examination revealed meningioma with intraosseous spread of tumor cells. Figure 3 shows photomicrographic examples of the intraosseous portion of tumor and the soft tumor.

MANAGEMENT
With the advent and characterization of new treatment
modalities, there are numerous management options in the
treatment of orbital meningiomas. Endovascular embolization can effectively devascularize meningiomas. Embolization is typically used in conjunction with open
resection of the tumor. Whole-brain irradiation and stereotactic radiosurgery have been shown to be effective in
controlling tumor growth. Radiosurgery can be applied
through the gamma knife or through a linear accelerator.
Radiosurgery has been used as a primary mode of treatment for intracranial meningiomas. In the skull base it
is frequently used as adjuvant therapy, either applied to
residual tumor postoperatively or to recurrent tumor.
Intraoperatively, the use of computer-assisted image guidance has helped in establishing the tumor borders and
localization. A different modification to cranial base approaches has assisted the surgeon in achieving a grosstotal resection without compromising neural structures. At
5

P. T. Boulos, et al.

Fig. 2. Intraoperative computer-assisted image guidance studies


obtained during osseous tumor resection. The intraoperative image
guidance screen is shown while in use. The crosshairs on the screen
correlate with the tip of the guidance probe. The probe is being
placed at the medial aspect of the sphenoid wing. The system allows visualization of the probe in three dimensions: coronal, sagittal, and axial. The images that are presented in this case are
T1-weighted MR images acquired after the administration of
gadolinium.

our institution, in cases of orbital meningiomas, we routinely obtain preoperative MR images and perform preoperative embolization. Intraoperative image guidance is
frequently used. Aggressive resection is attempted and
complete tumor removal is undertaken whenever safely
feasible. Radiosurgery is typically reserved for the treatment of patients in whom the tumors recur.
The treatment of optic nerve sheath meningiomas varies
from that of secondary orbital meningiomas. Whereas resection is the primary treatment modality for secondary
orbital meningiomas, patients with optic nerve sheath meningiomas are often followed clinically, undergoing visual field tests, visual acuity tests, and MR imaging studies.
This course of action is typically undertaken after the diagnosis is confirmed by examination of a biopsy sample.
Radiation therapy may slow the progression of the disease. As visual loss becomes progressive, or blindness has
ensued, surgery may be an option to relieve eye pain or
exophthalmos.
ENDOVASCULAR PREOPERATIVE
EMBOLIZATION
Embolization involves selective catheterization of the
blood vessels supplying the tumor. With the catheter in
6

Fig. 3. Photomicrographs. Upper: Tumor adjacent to dura.


Classical meningothelial architecture, with prominent lobulation
and whorl formation, is demonstrated. Lobules are surrounded by
collagenous septae. No atypical features can be seen. Lower: Intraosseous tumor. Extensive infiltration of the bone was noted,
with lobules of tumor invading osseous canaliculi. Infiltration of
skeletal muscle was also noted (data not shown). H & E, bar = 75
M (upper) and 150 M (lower).

place, embolic material is positioned within the lumen,


generating clot and thrombus. There has been some debate
as to whether embolization of meningiomas is a beneficial intervention. In 1973 Manelfe, et al.75 first described
the microcatheter technique of meningioma embolization.
Since that time many authors have demonstrated the potential benefits of embolizationnamely, decreased intraoperative blood loss, decreased complication rates, and
potentially reduced operative times.10,45,73,100,118 In many of
these studies the authors examined intracranial meningiomas in their entirety. It is clear that there are different
surgery-related considerations for meningiomas in different locations. Convexity meningiomas, in which the blood
supply is derived mainly from the external carotid artery
system, are less challenging in terms of endovascular embolization, yet on the other hand, intraoperative cauterization of the feeding vessels before tumor resection is also an effective technique for diminishing intraoperative
blood loss. Embolization is perhaps more helpful for selective cases such as when the surgeon must treat largesized meningiomas and those of the skull base.29,66,76,89 In
these cases the blood supply is derived from both the internal and external carotid systems and is not as easily surNeurosurg. Focus / Volume 10 / May, 2001

Orbital meningiomas
gically accessible. An orbital meningioma is a good example of a tumor that may derive its blood supply from
both the internal and external carotid systems. In cases in
which the tumors blood supply is derived from both systems, and only the external system is embolized, the benefit acheived remains controversial. Subjectively, we have
found that in the management of skull base osseous tumors preoperative embolization reduces the rate of intraoperative blood loss, allowing the surgeon to visualize the
tumor more effectively and work in an unhurried fashion.
SURGICAL APPROACHES
The surgical approach must be tailored to the individual
patient and his/her clinical status and pathological anatomy. Primarily intracranial lesions should be approached
intracranially, and anteriorly located orbital lesions should
be approached through the orbit.87
Transorbital access may be achieved using a variety of
approaches in which a lower-lid, subciliary, superior lid
crease, or an eyebrow incision is made. These provide different angles of approach to the anterior orbit. Many advances have been made over the years because of improved technology and accrued surgical experience. The
transorbital approach is typically used for lesions located
solely in the anterior orbit. An anteromedial microorbitotomy can be performed to treat lesions located in the
anteromedial orbit. A small operative field limits this
approach. A lateral orbitotomy permits retraction of the
globe laterally to expose the medial tumor, and this can
expand the operative field. For lesions located in the superior temporal or inferior compartment of the orbit a lateral orbitotomy can be performed. The extent of resection
from the lateral rim of the orbit bone and the sphenoid
ridge depends on the location of the tumor (superiorly
compared with inferiorly), as well as the depth of the
tumor. Currently Newman and Jane87 advocate the use of
a lateral orbitotomy in which a Burke incision is made.
Transcranially the orbit can be reached via a pterional,
frontotemporal, supraorbital ridge, or subfrontal approach.
A frontotemporal route can be used to approach primary
orbital tumors that extended intracranially. This approach
provides good exposure of the intraorbital contents, including the optic canal. This is achieved by excising the
orbital roof and the lateral orbital wall. The optic nerve
can be identified intradurally to facilitate the resection of
the orbital roof. The frontotemporal approach also provides good exposure of the anterior and middle cranial
fossae. A pterional approach can be used to reach sphenoid wing meningiomas that invade the orbit; this approach permits radical resection of the greater and lesser
wings of the sphenoid bone, as well as exposure of the lateral orbit, optic canal, and the supraorbital fissure, foramen rotundum, and foramen ovale. Fraizer39 first advocated the removal of the orbital rim to obtain additional
exposure of orbital contents. Jane and colleagues17,54 modified this approach by removing the supraorbital bone flap
in one piece. Colohan, et al.,20 introduced the concept of
using the frontal sinus to access the anterior cranial fossa
thereby providing better cosmesis.31 Jho56 has advocated
a minimally invasive approach: though an eyebrow incision, one can gain access to the posterior orbit and anteriNeurosurgical Focus / Volume 10 / May, 2001

or clinoid via an orbital roof craniotomy. Maroon, et al.78


have advocated a pterional approach and radical resection
of the greater and lesser wings of the sphenoid. They recommend decompressing the foramen rotundum, foramen
ovale, and the optic canal.
RADIATION THERAPY
Radiation therapy is a treatment option for meningiomas as a primary treatment modality or as adjuvant therapy. Wara, et al.,127 found in their 1975 series that adjuvant
postoperative radiotherapy decreased recurrence rates
from 74 to 29%. In several series and case reports authors
have reported an improvement in visual acuity in patients
in whom orbital meningiomas were treated primarily with
radiotherapy.63,109 One issue concerning irradiation as a
primary therapy is whether a tissue biopsy sample should
be obtained for diagnosis. Some authors have found that
inflammatory diseases can sometimes be misdiagnosed as
optic nerve sheath meningiomas.35 Others have suggested
that, at a minimum, a fine needle or open biopsy sampling
should be contemplated.19,77
Radiosurgery has become increasingly popular for the
treatment of meningiomas at many sites. In their series of
99 patients, Kondziolka, et al.,62 found that 63% of the tumors were reduced in size, 32% remained unchanged, and
5% enlarged. Resection was performed in 7% of patients
in whom there was continued neurological dysfunction or
continued tumor growth. Four of the tumors were located
at the sphenoid ridge. These patients were followed for
up to 10 years. In reviewing 62 patients with petroclival
meningiomas, Subach, et al.,116 found that tumor volumes
decreased in 23%, remained unchanged in 68%, and increased in 8%; the median follow-up period was 37
months. Hakim, et al.,46 examined 155 meningiomas treated with linear acceleratorbased radiosurgery and demonstrated that 89% had good tumor growth control at 5 years
posttreatment. Progression of disease was demonstrated in
15.7%. Radiosurgery is not without complications, however. Kalapurakal, et al.,57 reported on 42 patients treated
with radiotherapy, of whom 11 developed cerebral edema,
necessitating steroid therapy, and one patient developed
radiation-induced necrosis. In the series published by Subach, et al.,116 5 patients sustained cranial nerve palsies,
and one developed hydrocephalus. Although some success in controlling growth of meningiomas at multiple
sites in the calvarium, has been achieved with radiosurgery it remains to be seen if its application to orbital
meningiomas is efficacious and safe. The morbidity associated with orbital meningiomas is caused primarily by
compression of neural structures. It has yet to be determined if radiosurgery can sufficiently reduce tumor volume to relieve symptoms associated with orbital meningiomas.
PROGNOSIS
Meningiomas are thought to be a slow-growing but progressive tumors. The majority of recurrences are thought
to be due to residual tumor in the operative bed, which
happen because of fear of causing severe functional deficits, were complete resection attempted. Meningiomas
of all locations have a recurrence rates ranging from 10 to
7

P. T. Boulos, et al.
23%.1,82,119 Orbital meningiomas can invade the cavernous
sinus, the dura of the sella turcica, the lateral part of the
sphenoid body, the anulus of Zinn, the pterygomaxillary
fossa, and the lateropharyngeal space. In their report Bonnal, et al.,13 have argued against exploration of these areas
out of desire for a complete resection to preserve neurological function postoperatively. In their series of invading
meningiomas of the sphenoid ridge, residual tumor was
left in 18 of 21 cases for the aforementioned reasons. Recurrence rates of sphenoid ridge meningioma are much
higher than those located in other regions, ranging from
25 to 50%.1,85 Maroon and colleagues78 examined recurrent sphenoorbital meningiomas and found that they tended to be large infiltrating tumors that had a propensity
for invading vital neurological structures. They advocated
performing an extensive resection that includes the entire
greater and lesser wings of the sphenoid to the superior
orbital fissure, the anterior clinoid process, and the orbital
roof, as well as decompression of the foramen rotundum,
foramen ovale, and the optic canal. They do not recommend exploration of the cavernous sinus or the superior
orbital fissure.

9.
10.
11.
12.
13.
14.

15.
16.
17.
18.

CONCLUSIONS
The location of orbital meningiomas poses a particular
challenge to the surgeon. As such, residual tumor may be
left after resection, resulting in higher recurrence rates
than for meningiomas located in other regions. Fortunately, meningiomas are typically slow-growing benign
tumors that can be followed clinically and radiographically for recurrences. Should they recur, treatment options
remain. Radiation therapy has been shown to be effective
in controlling local tumor growth. Additional resection remains an option. A complete resection is still the most
desired result, as it may be curative. Current advances in
peri- and operative managementspecifically preoperative endovascular embolization, intraoperative computerassisted image guidance, and refined surgical approachesassist the surgeon in achieving optimum outcomes.

19.
20.
21.
22.
23.
24.
25.
26.

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Manuscript received April 10, 2001.


Accepted in final form May 7, 2001.
Address reprint requests to: Paul T. Boulos M.D., Box 800212
University of Virginia Health Sciences Center, Charlottesville, Virginia 22908. email: ptb8c@virginia.edu.

Neurosurg. Focus / Volume 10 / May, 2001