Meningiomas of the Orbit: Contemporary Considerations

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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 improv- ing 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 technology—namely, frameless computer-assisted image guidance—assist 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 assumed an important role in the evolution of the special- gratifying than the successful removal of a meningioma ty of neurological surgery. Some individuals have even with subsequent perfect functional recovery, especially asserted that meningiomas have had the most significant should a correct pathological diagnosis have been previ- impact in the development of our specialty.71,72 Mac- ously made. The difficulties are admittedly great, some- Carty71 wrote, “If we were to designate an intracranial times insurmountable, and though the disappointments neoplasm that has had the most effect on the development still are many, another generation of neurological sur- of neurologic surgery, very likely the intracranial menin- geons will unquestionably see them largely overcome.” – gioma would be prominently considered.” Harvey Cushing,26 1922. Meningiomas with orbital involvement have captured Meningiomas are the most common benign intracranial the interests of neurosurgeons, neurologists, and ophthal- lesions accounting for approximately 18% of all intracra- 98 mologists alike for over a century. Members from each of nial neoplasms. Meningiomas have kindled much inter- these specialties have contributed to the treatment of these est over the years because of their biological and clinical lesions. In the not-so-distant past, however, a collegial behavior and of the possibility of cure with appropriate 125 relationship did not always exist among practitioners. In therapy. Virchcow was the first to describe the classic 1938 Cushing reminisced, “[In] recent years, the ophthal- pathological feature of the meningioma—namely, the mic surgeon, the earliest surgical specialist, and the neu- psammoma body (“sandlike,” referring to the presence of rosurgeon, the latest, have from the opposite directions granules within the tumor). The nomenclature of these tumors evolved with time; numerous descriptions and con- come to meet at the barrier of the optic foramen—each somewhat hesitant to trespass on the other’s field of siderable ambiguity have been generated by scholars. To 27 eliminate such confusion Cushing26 in 1922 introduced work.” The successful treatment of these fascinating yet the simple yet all-encompassing term meningioma. Sub- often formidable lesions has required the harmonious sequently, meningiomas as a pathological entity have amalgamation of the expertise of ophthalmological and neurosurgical surgeons. Indeed, comprehensive and multidisciplinary cranial base teams have been assembled at Abbreviations used in this paper: CT = computerized tomogra- many institutions to treat patients harboring orbital me- phy; MR = magnetic resonance. ningiomas and other lesions of the cranial base.

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Meningiomas of the orbit can be thought of as primary ics meningioma.21,37 Suffice it to say that meningiomas are and secondary in origin. Primary orbital meningiomas overall uncommon in children (1.5 to 2.3% of all intracra- arise from the optic nerve sheath and may extend through nial tumors24); however, the relative incidence of orbital the optic canal intracranially, or they may infiltrate the involvement in childhood meningiomas has increased. sphenoid wing producing hyperostosis. Nearly two thirds Several groups have endeavored to determine the pre- of orbital tumors, however, originate from outside the disposing factors for orbital meningiomas. Neurofibroma- confines of the orbit.10,22,49,60,126 Secondary orbital menin- tosis appears to be one predisposing factor, particularly in giomas usually arise from the inner and outer aspects of pediatric meningiomas.47,58,67,83,88,106,108,120,126 Exposure to the sphenoid wing but not the middle sphenoid wing. radiation has clearly been associated with meningioma They may infiltrate medially into the orbit, often with formation (with a latency ranging from 12Ð27 years), intraorbital, intracranial, and intraosseous portions. They although there have been few cases in which orbital in- often compress the optic nerve by narrowing the optic volvement is described. The role of trauma and viral canal. They may infiltrate the contents of the supraorbital infection in the development of meningiomas is contro- fissure and cavernous sinus or compress the frontal and versial. 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 CLINICAL PRESENTATION wing have a relatively high incidence of bone involvement.95 It is thought that nearly 30% of orbital menin- The clinical presentation of orbital meningiomas has not changed since it was described by Cushing and giomas may involve bone (thus producing so-called hy- 27 perostosis), and 12% may originate from within the orbital Eisenhardt in 1938. Unilateral visual loss and progres- 102 sive, usually painless exophthalmos are the most com- bones. 21,97,105,124,131 The primary purpose of the present discussion is to ad- mon presentations described in the literature. Visual acuity tends to be involved early and exophthalmos dress comprehensively the unique topic of meningiomas 131 of the orbit. Data from the authors’ experience will be manifests later. The apparent visual loss generally de- included and the contemporary literature will be reviewed. velops gradually, although acute visual loss may occur in 2 to 5% of intracanalicular and 8 to 12% of intracranial Additionally, a recent case, complete with radiographic 128,129 and pathological data and intraoperative video clips will meningiomas. Other relatively common clinical fea- be used to illustrate many of the salient points germane to tures include optic disc changes, diplopia, headaches, and meningiomas of this region. 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 hyper- EPIDEMIOLOGY tension may produce papilledema in the contralateral eye. Meningiomas comprise approximately 18 to 20% of all Diplopia may be caused by cranial neuropathies or by intracranial tumors90 and are the most common benign in- direct disruption of the rectus muscles. Headache, as well tracranial neoplasm and the second most common intra- as nausea and vomiting, are typically associated with in- cranial tumor overall (second to only gliomas). Intracra- tracranial hypertension. Wright131 emphasized that visual nial meningiomas have been estimated to occur with an loss was the most common initial symptom, followed by incidence of 2.1/100,000 people.101 exophthalmos, diplopia, afferent papillary defect, and op- Meningiomas account for 3 to 9% of all orbital tu- tic nerve changes with shunt vessels present. So-called op- mors49,99 and are certainly not considered rare.81 Primary tociliary shunt vessels on the disc surface, representative orbital meningiomas represent between 0.4 to 2% of all of collateral circulation between the central retinal vein intracranial meningiomas.19,72,101 Secondary orbital menin- and the choroidal/ciliary venous drainage system,87 are giomas are considerably more common than their prima- strongly suggestive of meningiomas involving the anteri- ry counterparts. Sphenoid ridge meningiomas comprise or visual pathways. 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 PATHOLOGICAL CHARACTERISTICS Orbital meningiomas, as with those located in other re- The pathological origin of meningiomas is thought to gions, have a predilection for afflicting females (73 to be the arachnoid cap cells. The common sites for the tu- 84% occur in females19,131), although this female prepon- mor coincide with the presence of arachnoid villi, and derance does not appear to hold true in children with or- they are often tightly bound to areas of arachnoid granula- bital meningiomas30 and in meningiomas in general in tion tissue.4 Hence, the majority of meningiomas originate the pediatric population.34 Demographic data concerning in parasagittal, cavernous sinus, tuberculum sellae, sphe- the age distribution of meningiomas has been the sub- noid wing, lamina cribosa, foramen magnum, and torcular ject of some controversy. Although reported frequently in regions.18 Cushing must have appreciated this character- younger patients, particularly in the early literature,8,11,12, istic distribution, as he described meningiomas by their 28,33,52,79,80,122 others have found these tumors to be rela- common locations and clinical syndromes.26 tively uncommon in patients younger than 20 years of Meningiomas of the orbit have similar pathological age.53,58,68 Reports of orbital meningiomas in children may characteristics to other intracranial meningiomas. They have been erroneous in their diagnosis, as gliomas can exhibit various histological subtypes. Cushing and Eisen- evoke a surrounding dural reaction that superficially mim- hardt27 described nine main types and 20 subtypes. In the

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Unauthenticated | Downloaded 09/26/21 05:15 PM UTC Orbital meningiomas revised World Health Organization classification 15 sub- transitional with metaplastic changes that may be cartil- types are identified (Table 1). Meningothelial meningio- aginous, osseous, xanthomatous, myxoid, or lipomatous.65 mas are solid lobulated masses or sheets of meningothelial Atypical meningiomas deviate to some extent from the cells, in which the cell membrane is not well defined, and aforementioned benign features. They contain increased this gives an overall appearance of a syncytium; mitotic mitotic activity, increased cellularity, small cells, promi- activity is low. Fibrous meningiomas are composed of nent nucleoli, sheet-like growth, and areas of necrosis. bundles of cells that resemble fibroblasts; they typically Typically they are associated with a greater rate of recur- contain collagen and reticulin, and whorl formation and rence.16,65 Rhabdoid meningiomas are uncommon. They psammoma bodies may be focally present. Transitional are aggressive tumors that typically consist of patches or meningiomas are a mixture of the meningothelial and fi- sheets of rhabdoid cells. Rhabdoid cells are round with brous types.65 Psammomatous meningiomas contain many eccentric nuclei and prominent nucleoli. The cells also contain an eosinophilic cytoplasm with whorls of inter- psammoma bodies. Bone-related proteins, including os- 69 teopontin, are produced by CD68-positive macrophages mediate filaments. Papillary meningiomas are composed and may play a role in calcified psammoma body forma- of meningothelial cells in a radiating pattern resembling tion.50 Angiomatous meningiomas contain blood vessels pseudorosettes. They have a propensity for metastasis and of different shapes and sizes, typically with an endothelial recurrence. Anaplastic meningiomas have a more malig- lining. Microcystic meningiomas contain both macro- nant histological pattern than the atypical meningiomas. They contain higher mitotic activity and greater necrosis. scopic and intracellular microscopic cysts filled with eo- Their incidence is fortunately relatively low, ranging from sinophilic mucin; microcyst formation may be due to 0.9 to 10.6% in different series, with an overall mean rep- tumor cell secretions, tumor cell degeneration, cerebro- resentation of 2.8% of meningiomas.104 spinal fluid, penetration into the tumor, or vascular Primary orbital meningiomas arising from the optic changes. Secretory meningiomas are typically meningio- sheath typically consist of the transitional histological thelial or transitional lesions in which epithelial differen- subtype.2 In addition, psammomatous changes have been tiation has produced glandular structures. Clear cell me- noted in orbital meningiomas.23 Other histological sub- ningiomas contain sheets of polygonal cells with clear types including fibrous, papillary, and anaplastic menin- cytoplasm. The cytoplasm stains positive for periodic ac- giomas typically do not involve the orbit primarily.103 In idÐSchiff because of the presence of glycogen. Chordoid our evolving understanding of the development of optic meningiomas are composed of eosinophilic, vacuolated sheath meningiomas we have witnessed the replacement cells in trabeculae, resembling chordomas. Lymphoplas- of one hypothesis—that they arise primarily extradurally macyte-rich meningiomas are typically meningothelial, with secondary invasion of the optic dural sheath123—with fibrous, or transitional with a lymphocytic infiltrate. Me- another—that they arise from the dural sheath itself.22 taplastic meningiomas are meningothelial, fibrous, or These lesions then spread along the optic nerve, often en- veloping the nerve completely and thereby encompassing the pial blood supply.5 The optic nerve may be compro- mised by direct invasion by tumor or by compression TABLE 1 leading to atrophy.96,107 Furthermore, tumor extension be- World Health Organization classification of meningiomas* yond the dura may affect the extraocular muscles.110 Type of Meningioma WHO Grade As previously mentioned, meningiomas with orbital involvement, particularly those originating from the sphe- benign† noid wing, commonly demonstrate involvement of the meningothelial I bone. Osseous involvement may consist of hyperostosis fibrous (fibroblastic) I associated with an extraosseous meningioma, or the tumor transitional (mixed) I psammomatous I may originate within the bone itself. angiomatous I Hyperostosis associated with intracranial meningiomas microcystic I is a well-recognized phenomenon. The reported incidence secretory I varies from 2525Ð27 to 49%.40,91,111 Numerous theories have lymphoplasmacyte-rich I emerged to account for hyperostosis and include vascular metaplastic I disturbances of the bone induced by the tumor,13,41,48,64,86,92 aggressive‡ 3,7,9,27,42,92,112,113,115,117 atypical II preceding trauma, osseous reaction to 25,59,93 clear cell (intracranial) II the adjacent tumor without tumor invasion, produc- chordoid II tion of bone by the tumor itself,41,48,64,86,92 stimulation of rhabdoid III osteoblasts in adjacent normal bone by tumor-secreted papillary III factors,36,44,78,95 and tumor invasion of the bone.13,15,29,36, anaplastic III 44,61,78,95 Recent and commendable work reported by Al- * Adapted from Louis DN, Scheithauer BW, Budka H, et al: Menin- Mefty’s group lends credence to the hypothesis that hy- 94 giomas, in Kleihues P, Cavenee WK (eds): Pathology and Genetics of Tu- perostosis results from tumor invasion of the bone. mours of the Nervous System. Lyon: IARC Press, 2000, 176Ð184. These authors demonstrated this finding through careful Abbreviation: WHO = World Health Organization. radiographic and surgical/histopathological correlation of † Defined as those with a low risk of recurrence and aggressive growth meningiomas involving the cranial base. The origin of behavior. ‡ Defined as those with a greater risk of recurrence and aggressive hyperostosis is important in the surgical management of growth behavior. meningiomas. Given that hyperostosis is likely secondary

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Unauthenticated | Downloaded 09/26/21 05:15 PM UTC P. T. Boulos, et al. to tumor invasion and that complete tumor removal is the TABLE 2 desired goal, resection of the hyperostotic bone becomes Differential diagnosis for orbital lesions mandatory. apparent origin—optic nerve tumors RADIOLOGICAL FINDINGS meningioma (from the optic nerve sheath) glioma (benign or malignant) Optic nerve sheath meningiomas may appear normal on neuroma/neurofibroma (plexiform neuroma w/ neurofibromatosis) plain x-ray films in the early stages of tumor development. metastatic (lung/breast/prostate cancer, leukemic infiltration, Late in tumor development, optic canal enlargement or neuroblastoma, Ewing tumor hyperostosis of the optic canal may be observed. Com- other (arachnoid cysts, juvenile xanthogranulomas) puterized tomography scanning of specifically optic nerve inflammatory conditions multiple sclerosis optic neuritis sheath meningiomas may reveal thickening of the optic infectious optic neuritis (syphilis, viral) nerve or the “tram track sign” in which two strips of lu- other (sarcoidosis) cency are seen around the center of the enlarged optic vascular conditions nerve, signifying that the optic nerve is surrounded by central retinal vein occlusion tumor.74 This feature is in contrast to optic nerve gliomas apparent origin—adjacent regions in which the optic nerve itself is expanded.105 The pres- meningioma (sphenoid wing, cavernous sinus, suprasellar) glioma ence of calcification is an important feature that is highly cavernous hemangioma suggestive of meningioma. Hyperostosis of the optic canal hemangioblastoma may also be evident on CT scans. Contrast administration hemangiopericytoma typically reveals homogeneous enhancement of the mass. fibrous dysplasia Magnetic resonance imaging can provide more details bone tumor about the tumor; specifically it can help differentiate fu- infectious process (abscess) aneurysm siform lesions and loculated from eccentric lesions. Ad- malignant sinonasal tumor (squamous cell carcinoma, rhabdo- ditionally MR imaging can also detect smaller lesions. En myosarcoma) plaque lesions can be seen on gadolinium-enhanced fat- metastasis dermoid tumor suppressed T1-weighted MR images as two strips of enhancement on either side of the optic nerve.74 benign mixed lacrimal gland/mucocele Either hyperostosis or erosion of the orbital bone may cholesteatoma venous varix be seen on radiographs of Sphenoid ridge meningiomas. arteriovenous malformation Computerized tomography examination with and without nonspecific orbital inflammation contrast medium can often reveal the extent of the tumor’s intracranial involvement into the anterior and middle cranial fossae. It can also further delineate the involvement of the bone with hyperostosis or tumor infiltration. Ad- originally thought, based on anecdotal reports, that menin- ditionally, CT scanning can demonstrate the extent of in- giomas affecting the orbit did not portend a clinical course traorbital involvement. Magnetic resonance imaging can of progression and neurological deterioration.6,38,114 As ex- provide finer detail of the anatomical location of the perience has accrued and long-term follow-up data have tumor. A clear dural tail, which would support the diag- become available, however, it has become apparent that nosis of meningioma, may also be visualized using MR these tumors almost invariably progress43,51,70,105,106,121 and imaging. may grow to overwhelming size with time.55 As mentioned previously, orbital meningiomas afflicting pediatric patients may be associated with a poorer DIFFERENTIAL DIAGNOSIS prognosis than in older patients.2,84,126 Analysis of some data suggests that in the pediatric population, these tumors An understanding of orbital anatomy facilitates the for- may not only produce visual impairment but also may mulation of a differential diagnosis for orbital menin- extend to adjacent areas such as the cavernous sinus, sel- giomas. Lesions may arise from the optic nerve and its lar region, the anulus of Zinn, and the pterygomaxillary adjacent structures (Table 2). A conglomeration of path- fossa,13 particularly with tumor involvement of the medi- ological entities may affect the optic nerve and surround- al sphenoid wing.14 With tumor extension, complete ex- ing orbital contents. Enlargement of the optic nerve may, tirpation without significant neurological compromise for example, be due to tumor involvement in addition to a becomes exceedingly difficult.32 multitude of inflammatory conditions or vascular condi- 87 Thus it appears that meningiomas with orbital involve- tions. Masses arising from the surrounding vicinity may ment progress, leading to potential neurological compro- also result from a host of conditions. mise. This is particularly so in the younger population in whom these tumors appear to behave more aggressively. NATURAL HISTORY Rational treatment decisions regarding the treatment of ILLUSTRATIVE CASE orbital meningiomas are predicated on an appreciation of the natural history of the disease as well as a detailed History and Examination. This 35-year-old right-hand- understanding of the available treatment modalities and ed woman presented to an ophthalmologist with a history their associated benefits, limitations, and risks. It was of right eye swelling and exophthalmos. She complained

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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 less guidance system is calibrated to the skull with a rigid physical examination right-sided exophthalmos was ob- fixation system. Over the course of the operation the served, yet otherwise she was neurologically intact. Find- osseous structures do not shift with retraction or decom- ings on cranial nerve and sensorimotor examinations were pression; thus, the skull base was believed to be accurate within normal limits. (Fig. 2). Peroperative Treatment. The patient underwent MR imaging and CT examination. As seen in Fig. 1 left and VIDEO CLIP 2. Soft tumor removal. This is an intraoperative center, these studies revealed an enhancing lesion anterior video demonstrating the removal of the intracranial soft tumor, to the tip of the right temporal lobe, as well as widening of which was performed in a standard fashion by using bipolar elec- the sphenoid ridge with extension of the intraosseous trocautery and microinstruments. mass into the orbit. Endovascular embolization was performed. As seen in Fig. 1 right, external carotid artery an- Postoperative Course. The patient awoke from anesthe- giography revealed a tumor blush, as is frequently seen in sia without cranial nerve dysfunction. After postoperative meningiomas. The tumor was embolized through selec- swelling had subsided, the exophthalmos resolved. Final tive catheterization of the middle meningial artery. There pathological examination revealed meningioma with in- was no angiographic evidence of the tumor’s blood supply traosseous spread of tumor cells. Figure 3 shows photo- being derived from the internal carotid artery system. micrographic examples of the intraosseous portion of tu- Operation. The patient was taken to the operating room mor and the soft tumor. the day after endovascular embolization. A frontotemporal craniotomy was performed. The tumor portion extend- ing into the sphenoid ridge was resected in an extradural MANAGEMENT fashion. As seen in Video Clip 1, the lateral wall of the With the advent and characterization of new treatment orbit was removed to the anterior clinoid process. The soft modalities, there are numerous management options in the intraorbital portion of the tumor was then removed. treatment of orbital meningiomas. Endovascular embolization can effectively devascularize meningiomas. Em- VIDEO CLIP 1. Osseous bone removal. This is an intraoperative bolization is typically used in conjunction with open video edited to depict several key points of the osseous removal. A resection of the tumor. Whole-brain irradiation and stereo- variety of clips demonstrates drilling of the sphenoid ridge. The tactic radiosurgery have been shown to be effective in drill is used to thin the tumor-infiltrated lateral wall of the orbit. controlling tumor growth. Radiosurgery can be applied The punch is then used to remove the thinned wall to the orbit. The through the gamma knife or through a linear accelerator. final frames demonstrate the absence of the greater and lesser sphe- Radiosurgery has been used as a primary mode of treat- noid ridges, as well as the contents of the supraorbital fissure left intact. ment for intracranial meningiomas. In the skull base it is frequently used as adjuvant therapy, either applied to residual tumor postoperatively or to recurrent tumor. As demonstrated in Video Clip 2, the intradural portion Intraoperatively, the use of computer-assisted image guid- of the tumor was then removed. The procedure was per- ance has helped in establishing the tumor borders and formed with the assistance of computer image guidance, localization. A different modification to cranial base ap- which was found to be particularly useful in this case proaches has assisted the surgeon in achieving a gross- because much of the tumor was intraosseous. The frame- total resection without compromising neural structures. At

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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 al- Fig. 3. Photomicrographs. Upper: Tumor adjacent to dura. lows visualization of the probe in three dimensions: coronal, sag- Classical meningothelial architecture, with prominent lobulation ittal, and axial. The images that are presented in this case are and whorl formation, is demonstrated. Lobules are surrounded by collagenous septae. No atypical features can be seen. Lower: In- T1-weighted MR images acquired after the administration of gadolinium. traosseous 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). our institution, in cases of orbital meningiomas, we rou- tinely obtain preoperative MR images and perform preoperative embolization. Intraoperative image guidance is place, embolic material is positioned within the lumen, frequently used. Aggressive resection is attempted and generating clot and thrombus. There has been some debate complete tumor removal is undertaken whenever safely as to whether embolization of meningiomas is a benefi- feasible. Radiosurgery is typically reserved for the treat- cial intervention. In 1973 Manelfe, et al.75 first described ment of patients in whom the tumors recur. the microcatheter technique of meningioma embolization. The treatment of optic nerve sheath meningiomas varies Since that time many authors have demonstrated the po- from that of secondary orbital meningiomas. Whereas re- tential benefits of embolization—namely, decreased intra- section is the primary treatment modality for secondary operative blood loss, decreased complication rates, and orbital meningiomas, patients with optic nerve sheath me- potentially reduced operative times.10,45,73,100,118 In many of ningiomas are often followed clinically, undergoing visu- these studies the authors examined intracranial meningial field tests, visual acuity tests, and MR imaging studies. omas in their entirety. It is clear that there are different This course of action is typically undertaken after the di- surgery-related considerations for meningiomas in differ- agnosis is confirmed by examination of a biopsy sample. ent locations. Convexity meningiomas, in which the blood Radiation therapy may slow the progression of the dis- supply is derived mainly from the external carotid artery ease. As visual loss becomes progressive, or blindness has system, are less challenging in terms of endovascular em- ensued, surgery may be an option to relieve eye pain or bolization, yet on the other hand, intraoperative cauteri- exophthalmos. zation of the feeding vessels before tumor resection is also an effective technique for diminishing intraoperative ENDOVASCULAR PREOPERATIVE blood loss. Embolization is perhaps more helpful for se- EMBOLIZATION lective cases such as when the surgeon must treat large- sized meningiomas and those of the skull base.29,66,76,89 In Embolization involves selective catheterization of the these cases the blood supply is derived from both the in- blood vessels supplying the tumor. With the catheter in ternal and external carotid systems and is not as easily sur-

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Unauthenticated | Downloaded 09/26/21 05:15 PM UTC Orbital meningiomas gically accessible. An orbital meningioma is a good ex- or clinoid via an orbital roof craniotomy. Maroon, et al.78 ample of a tumor that may derive its blood supply from have advocated a pterional approach and radical resection both the internal and external carotid systems. In cases in of the greater and lesser wings of the sphenoid. They rec- which the tumor’s blood supply is derived from both sys- ommend decompressing the foramen rotundum, foramen tems, and only the external system is embolized, the ben- ovale, and the optic canal. efit acheived remains controversial. Subjectively, we have found that in the management of skull base osseous tumors preoperative embolization reduces the rate of intra- RADIATION THERAPY operative blood loss, allowing the surgeon to visualize the Radiation therapy is a treatment option for meningio- tumor more effectively and work in an unhurried fashion. mas 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 SURGICAL APPROACHES from 74 to 29%. In several series and case reports authors have reported an improvement in visual acuity in patients The surgical approach must be tailored to the individual in whom orbital meningiomas were treated primarily with patient and his/her clinical status and pathological anato- radiotherapy.63,109 One issue concerning irradiation as a my. Primarily intracranial lesions should be approached primary therapy is whether a tissue biopsy sample should intracranially, and anteriorly located orbital lesions should 87 be obtained for diagnosis. Some authors have found that be approached through the orbit. inflammatory diseases can sometimes be misdiagnosed as Transorbital access may be achieved using a variety of optic nerve sheath meningiomas.35 Others have suggested approaches in which a lower-lid, subciliary, superior lid that, at a minimum, a fine needle or open biopsy sampling crease, or an eyebrow incision is made. These provide dif- should be contemplated.19,77 ferent angles of approach to the anterior orbit. Many ad- Radiosurgery has become increasingly popular for the vances have been made over the years because of im- treatment of meningiomas at many sites. In their series of proved technology and accrued surgical experience. The 99 patients, Kondziolka, et al.,62 found that 63% of the tu- transorbital approach is typically used for lesions located mors were reduced in size, 32% remained unchanged, and solely in the anterior orbit. An anteromedial microorbi- 5% enlarged. Resection was performed in 7% of patients totomy can be performed to treat lesions located in the in whom there was continued neurological dysfunction or anteromedial orbit. A small operative field limits this continued tumor growth. Four of the tumors were located approach. A lateral orbitotomy permits retraction of the at the sphenoid ridge. These patients were followed for globe laterally to expose the medial tumor, and this can up to 10 years. In reviewing 62 patients with petroclival expand the operative field. For lesions located in the supe- meningiomas, Subach, et al.,116 found that tumor volumes rior temporal or inferior compartment of the orbit a later- decreased in 23%, remained unchanged in 68%, and in- al orbitotomy can be performed. The extent of resection creased in 8%; the median follow-up period was 37 from the lateral rim of the orbit bone and the sphenoid months. Hakim, et al.,46 examined 155 meningiomas treat- ridge depends on the location of the tumor (superiorly ed with linear acceleratorÐbased radiosurgery and demon- compared with inferiorly), as well as the depth of the 87 strated that 89% had good tumor growth control at 5 years tumor. Currently Newman and Jane advocate the use of posttreatment. Progression of disease was demonstrated in a lateral orbitotomy in which a Burke incision is made. 15.7%. Radiosurgery is not without complications, how- Transcranially the orbit can be reached via a pterional, ever. Kalapurakal, et al.,57 reported on 42 patients treated frontotemporal, supraorbital ridge, or subfrontal approach. with radiotherapy, of whom 11 developed cerebral edema, A frontotemporal route can be used to approach primary necessitating steroid therapy, and one patient developed orbital tumors that extended intracranially. This approach radiation-induced necrosis. In the series published by Su- provides good exposure of the intraorbital contents, in- bach, et al.,116 5 patients sustained cranial nerve palsies, cluding the optic canal. This is achieved by excising the and one developed hydrocephalus. Although some suc- orbital roof and the lateral orbital wall. The optic nerve cess in controlling growth of meningiomas at multiple can be identified intradurally to facilitate the resection of sites in the calvarium, has been achieved with radio- the orbital roof. The frontotemporal approach also pro- surgery it remains to be seen if its application to orbital vides good exposure of the anterior and middle cranial meningiomas is efficacious and safe. The morbidity asso- fossae. A pterional approach can be used to reach sphe- ciated with orbital meningiomas is caused primarily by noid wing meningiomas that invade the orbit; this ap- compression of neural structures. It has yet to be deter- proach permits radical resection of the greater and lesser mined if radiosurgery can sufficiently reduce tumor vol- wings of the sphenoid bone, as well as exposure of the lat- ume to relieve symptoms associated with orbital menin- eral orbit, optic canal, and the supraorbital fissure, fora- giomas. men rotundum, and foramen ovale. Fraizer39 first advocated the removal of the orbital rim to obtain additional exposure of orbital contents. Jane and colleagues17,54 mod- PROGNOSIS ified this approach by removing the supraorbital bone flap Meningiomas are thought to be a slow-growing but pro- in one piece. Colohan, et al.,20 introduced the concept of gressive tumors. The majority of recurrences are thought using the frontal sinus to access the anterior cranial fossa to be due to residual tumor in the operative bed, which thereby providing better cosmesis.31 Jho56 has advocated happen because of fear of causing severe functional de- a minimally invasive approach: though an eyebrow inci- ficits, were complete resection attempted. Meningiomas sion, one can gain access to the posterior orbit and anteri- of all locations have a recurrence rates ranging from 10 to

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23%.1,82,119 Orbital meningiomas can invade the cavernous tique chez un enfant de sept ans. Rev Oto Neurol Ophthalmol sinus, the dura of the sella turcica, the lateral part of the 30:486Ð489, 1958 sphenoid body, the anulus of Zinn, the pterygomaxillary 9. Ashhurst APC: Case of jacksonian epilepsy caused by brain fossa, and the lateropharyngeal space. In their report Bon- tumor. Ann Surg 72:402Ð406, 1920 13 10. Bendszus M, Rao G, Burger R, et al: Is there a benefit of nal, et al., have argued against exploration of these areas preoperative meningioma embolization? Neurosurgery 47: out of desire for a complete resection to preserve neuro- 1306Ð1312, 2000 logical function postoperatively. In their series of invading 11. Benedict WL: Surgical treatment of tumors and cysts of the or- meningiomas of the sphenoid ridge, residual tumor was bit. Am J Ophthalmol 32:763Ð773, 1949 left in 18 of 21 cases for the aforementioned reasons. Re- 12. Benedict WL: Tumors and cysts arising near apex of orbit. Am currence rates of sphenoid ridge meningioma are much J Ophthalmol 6:183Ð201, 1923 higher than those located in other regions, ranging from 13. Bonnal J, Thibaut A, Brotchi J, et al: Invading meningiomas of 25 to 50%.1,85 Maroon and colleagues78 examined recur- the sphenoid ridge. J Neurosurg 53:587Ð599, 1980 rent sphenoorbital meningiomas and found that they tend- 14. Bonnal JP, Brotchi J, Born J: Meningiomas of the sphenoid ed to be large infiltrating tumors that had a propensity wings, in Sehkar LN, Schramm VL Jr (eds): Tumors of the Cranial Base: Diagnosis and Treatment. Mount Kisco, NY: for invading vital neurological structures. They advocated Futura, 1987, pp 373Ð392 performing an extensive resection that includes the entire 15. Castellano F, Guidetti B, Olivecrona H: Pterional meningiomas greater and lesser wings of the sphenoid to the superior "en plaque". J Neurosurg 9:188Ð196, 1952 orbital fissure, the anterior clinoid process, and the orbital 16. Chen WY, Liu HC: Atypical (anaplastic) meningioma: rela- roof, as well as decompression of the foramen rotundum, tionship between histologic features and recurrence—a clinico- foramen ovale, and the optic canal. They do not recom- pathologic study. Clin Neuropathol 9:74Ð81, 1990 mend exploration of the cavernous sinus or the superior 17. Chenelle AG, Shaffery ME, Delashaw JB Jr, et al: Neurosurgi- orbital fissure. cal considerations of cranial base surgery. Clin Plastic Surg 22:451Ð460, 1995 18. Chou SM, Miles JM: The pathology of meningiomas, in Al- Mefty O (ed): Meningiomas. New York: Raven Press, 1991, CONCLUSIONS pp 37Ð57 19. Clark WC, Theofilos CS, Fleming JC: Primary optic nerve The location of orbital meningiomas poses a particular sheath meningiomas. Report of nine cases. J Neurosurg 70: challenge to the surgeon. As such, residual tumor may be 37Ð40, 1989 left after resection, resulting in higher recurrence rates 20. Colohan AR, Jane JA, Newman SA, et al: Frontal sinus ap- than for meningiomas located in other regions. Fortu- proach to the orbit. Technical note. J Neurosurg 63:811Ð 813, nately, meningiomas are typically slow-growing benign 1985 tumors that can be followed clinically and radiographical- 21. Cooling RJ, Wright JE: Arachnoid hyperplasia in optic nerve ly for recurrences. Should they recur, treatment options glioma: confusion with orbital meningioma. Br J Ophthalmol remain. Radiation therapy has been shown to be effective 63:596Ð599, 1979 in controlling local tumor growth. Additional resection re- 22. Craig W McK, Gogela LJ: Intraorbital meningiomas. A clinico- mains an option. A complete resection is still the most pathologic study. Am J Ophthalmol 32:1663Ð1680, 1949 23. Craig W McK, Gogela LJ: Meningioma of the optic foramen as desired result, as it may be curative. Current advances in a cause of slowly progressive blindness. Report of 3 cases. J peri- and operative management—specifically preopera- Neurosurg 7:44Ð48, 1950 tive endovascular embolization, intraoperative computer- 24. Crouse SK, Berg BO: Intracranial meningiomas in childhood assisted image guidance, and refined surgical approach- and adolescence. Neurology 22:135Ð141, 1972 es—assist the surgeon in achieving optimum outcomes. 25. Cushing H: The cranial hyperostoses produced by meningeal endotheliomas. Arch Neurol Psychiatry 8:139Ð154, 1922 26. Cushing H: The meningiomas (dural endotheliomas): their References source, and favoured seats of origin. Brain 45:282Ð316, 1922 27. Cushing H, Eisenhardt L: Meningiomas: Their Classification, 1. Adegbite AB, Khan MI, Paine KW, et al: The recurrence of Regional Behaviour, Life History, and Surgical End Re- intracranial meningiomas after surgical treatment. J Neurosurg sults. Springfield, IL: Charles C Thomas, 1938 58:51Ð56, 1983 28. Dandy WE: Prechiasmal intracranial tumors of the optic nerves. 2. Alper MG: Management of primary optic nerve meningiomas. Am J Ophthalmol 5:169Ð188, 1922 Current status—therapy in controversy. J Clin Neuroophthal- 29. Dean BL, Flom RA, Wallace RC, et al: Efficacy of endovascu- mol 1:101Ð117, 1981 lar treatment of meningiomas: evaluation with matched sam- 3. Alpers BJ, Harow R: Cranial hyperostosis. Arch Neurol Psy- ples. AJNR 15:1675Ð1680, 1994 chiatry 28:339Ð356, 1932 30. Deen HG Jr, Scheithauer BW, Ebersold MJ: Clinical and patho- 4. Al-Rodhan NRF, Laws ER Jr: The history of intracranial me- logical study of meningiomas in the first two decades of life. J ningiomas, in Al-Mefty O (ed): Meningiomas. New York: Neurosurg 56:317Ð322, 1982 Raven Press, 1991, pp 1Ð7 31. Delashaw JB Jr, Tedeschi H, Rhoton AL: Modified supraorbital 5. Anderson DR, Hoyt WF: Ultrastructure of intraorbital portion craniotomy: technical note. Neurosurgery 30:954Ð956, 1992 of human and monkey optic nerve. Arch Opthalmol 82: 32. Derome PJ, Guiot G: Bone problems in meningiomas invading 506Ð530, 1969 the base of the skull. Clin Neurosurg 25:435Ð451, 1978 6. Arbit E: Optic nerve sheath meningioma. J Neurosurg 71:151, 33. de Schweinitz GE: Psammosarcoma of the orbit in a girl of thir- 1989 (Letter) teen. Successful removal with preservation of the eyeball and 7. Archibald E: Surgical affections and wounds of the head, in its functions. Trans Am Ophthalmol Soc 13:770Ð777, 1914 Bryant J, Buck A (eds): American Practice of Surgery. New 34. Doty JR, Schut L, Bruce DA, et al: Intracranial meningiomas of York, William Wood, 1908, Vol 4, pp 3Ð378 childhood and adolescence. Prog Exp Tumor Res 30: 8. Ardouin M, Pecker J, Stabert C, et al: Méningiome du canal op- 247Ð254, 1987

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35. Dutton JJ, Anderson RL: Idiopathic inflammatory perioptic tic sign of hyperostosing meningioma en plaque. AJR 141: neuritis simulating optic nerve sheath meningioma. Am J Oph- 1217Ð1221, 1983 thalmol 100:424Ð430, 1985 62. Kondziolka D, Levy EI, Niranjan A, et al: Long-term outcomes 36. Echlin F: Cranial osteomas and hyperostoses produced by men- after meningioma radiosurgery: physician and patient perspec- ingeal fibroblastomas. Arch Surg 28:357Ð405, 1934 tives. J Neurosurg 91:44Ð50, 1999 37. Editorial: Primary optic nerve meningioma Br J Ophthalmol 63. Kupersmith MJ, Warren FA, Newall J, et al: Irradiation of me- 63:595, 1979 ningiomas of the intracranial anterior visual pathway. Ann 38. Ellenberger C: Perioptic meningiomas. Syndrome of long- Neurol 21:131Ð137, 1987 standing visual loss, pale disk edema, and optociliary veins. 64. Landow H, Kabat EA, Newman W: Distribution of alkaline Arch Neurol 33:671Ð674, 1976 phophatase in normal and neoplastic tissues of the nervous sys- 39. Frazier CH: An approach to the hypophysis through the anteri- tem. Arch Neurol Psychiatry 48:518Ð530, 1942 or cranial fossa. Ann Surg 57:145Ð 152, 1913 65. Lantos PL, VandenBerg SR, Kleihues P: Tumours of the ner- 40. Frazier CH, Alpers BJ: Meningeal fibroblastomas of the cere- vous system, in Graham DI, Lantos PL (eds): Greenfield's brum. Arch Neurol Psychiatry 29:935Ð989, 1933 Neuropathology, ed 6. New York: Oxford University Press, 41. Freeman H, Forster FM: Bone formation and destruction in 1997, Vol 2, pp 583Ð880 hyperostoses associated with menigiomas. J Neuropathol Exp 66. Latchaw RE: Preoperative intracranial meningioma emboliza- Neurol 7:69Ð80, 1948 tion: technical considerations affecting the risk-to-benefit ratio. 42. Fuchs: A successfully operated case of brain tumor. Wien Klin AJNR 14:583Ð586, 1993 Wochenschr 23:1701, 1910 67. Liano H, Garcia-Alix C, Lousa M, et al: Bilateral optic nerve 43. Gabibov GA, Blinkov SM, Tcherekayev VA: The management meningioma. Case report. Eur Neurol 21:102Ð106, 1982 of optic nerve meningiomas and gliomas. J Neurosurg 68: 68. Lloyd GA: Primary orbital meningioma: a review of 41 patients 889Ð893, 1988 investigated radiologically. Clin Radiol 33:181Ð187, 1982 44. Globus JH: The meningiomas. Trans Assoc Res Nerv Ment 69. Louis DN, Scheithauer BW, Budka H, et al: Meningiomas, in Dis 16:210Ð265, 1937 Kleihues P, Cavenee WK (eds): Pathology and Genetics of 45. Gruber A, Killer M, Mazal P, et al: Preopertive embolization of Tumours of the Nervous System. Lyon: IARC Press, 2000, intracranial meningiomas: a 17-year single center experience. 176Ð184 Minim Invasive Neurosurg 43:18Ð29, 2000 70. Love JG, Rucker CW: Meningioma of the sheath of the optic 46. Hakim R, Alexander E III, Loeffler JS, et al: Results of linear nerve. Report of a case. Arch Ophthalmol 23:377Ð380, 1940 accelerator-based radiosurgery for intracranial meningiomas. 71. MacCarty CS: The Surgical Treatment of Intracranial Me- Neurosurgery 42:446Ð454, 1998 ningiomas. Springfield, IL: Charles C Thomas, 1961 47. Hart WM Jr, Burde RM, Klingele TG, et al: Bilateral optic 72. MacCarty CS, Piepgras DG, Ebersold MJ: Meningeal tumors of sheath meningiomas. Arch Ophthalmol 98:149Ð151, 1980 the brain, in Youmans JR (ed): Neurological Surgery, ed 2. 48. Heick A, Mosdal C, Jorgensen K, et al: Localized cranial hyper- Philadelphia: WB Saunders, 1982, Vol 5, pp 2936Ð2966 ostosis of meningiomas: a result of neoplastic enzyme activity? 73. Macpherson P: The value of pre-operative embolisation of me- Acta Neurol Scand 87:243Ð247, 1993 ningioma estimated subjectively and objectively. Neuroradi- 49. Henderson JW, Farrow GM: Orbital Tumors, ed 2. New York: ology 33:334Ð337, 1991 BC Decker, 1980, pp 472Ð496 74. Mafee MF, Goodwin J, Dorodi S: Optic nerve sheath menin- 50. Hirota S, Nakajima Y, Yoshimine T, et al: Expression of bone- giomas. Role of MR imaging. Radiol Clin North Am 37: related protein messenger RNA in human meningiomas: possi- 37Ð58, 1999 ble involvement of osteopontin in development of psammoma 75. Manelfe C, Guiraud B, David J, et al: [Embolization by cath- bodies. J Neuropathol Exp Neurol 54:698Ð703, 1995 eterization of intracranial meningiomas.] Rev Neurol 128: 51. Hollenhorst RW Jr, Hollenhorst RW Sr, MacCarty CS: Visual 339Ð351, 1973 (Fr) prognosis of optic nerve sheath meningiomas producing shunt 76. Manelfe C, Lasjaunias P, Ruscalleda J: Preoperative emboliza- vessels on the optic disk: the HoytÐSpencer syndrome. Trans tion of intracranial meningiomas. AJNR 7:963Ð972, 1986 Am Ophthalmol Soc 75:141Ð163, 1977 77. Maroon JC, Abla A, Kennerdell JS, et al: Orbital tumor, in Long 52. Hudson AC: Primary tumours of the optic nerve. Roy London DM (ed): Current Therapy in Neurological Surgery—2. Ophthalmol Hosp Rep 18:317Ð439, 1912 Philadelphia: BC Decker, 1989, pp 29Ð36 53. Jakobiec FA, Depot MJ, Kennerdell JS, et al: Combined clini- 78. Maroon JC, Kennerdell JS, Vidovich DV, et al: Recurrent sphe- cal and computed tomographic diagnosis of orbital glioma and no-orbital meningioma. J Neurosurg 80:202Ð208, 1994 meningioma. Ophthalmology 91:137Ð155, 1984 79. Martin VA, Schofield PB: Meningioma invading the optic 54. Jane JA, Park TS, Pobereskin LH, et al: The supraorbital ap- nerve. Br J Ophthalmol 41:161Ð166, 1957 proach: technical note. Neurosurgery 11:537Ð542 1982 80. Mayer LL: Endothelioma of orbit. Report of case. Am J Oph- 55. Jensen IK: Orbital meningioma. A monstrous case with a 30 thalmol 11:617Ð622, 1928 years history of the disease. Acta Ophthalmol 44:684Ð688, 81. Mehra KS, Khanna S, Dube B: Primary meningioma of the 1966 intraorbital optic nerve. Ann Ophthalmol 11:758Ð760, 1979 56. Jho HD: Orbital roof craniotomy via an eyebrow incision: a 82. Melamed S, Sahar A, Beller AJ: The recurrence of intracranial simplified anterior skull base approach. Minim Invasive Neu- meningiomas. Neurochirurgia 22:47Ð51, 1979 rosurg 40:91Ð97, 1997 83. Merten DF, Gooding CA, Newton TH, et al: Meningiomas of 57. Kalapurakal JA, Silverman CL, Akhtar N, et al: Intracranial childhood and adolescence. J Pediatr 84:696Ð700, 1974 meningiomas: factors that influence the development of cere- 84. Miller NR: Progressive visual loss. What type of mass lesion? bral edema after stereotactic radiosurgery and radiation therapy. Surv Ophthalmol 28:45Ð49, 1983 Radiology 204:461Ð465, 1997 85. Mirimanoff RO, Dosoretz DE, Linggood RM, et al: analysis of 58. Karp LA, Zimmerman LE, Borit A, et al: Primary intraorbital recurrence and progression following neurosurgical resection. J meningiomas. Arch Ophthalmol 91:24Ð28, 1974 Neurosurg 62:18Ð24, 1985 59. Kennedy F: Tumours of the intracranial cavity, in Nelson (ed): 86. Nasu H: Ferment histochemische untersuchungen an meninge- Loose Leaf Living Medicine. London, 1920, pp 119Ð136 omen. Acta Neuropathol 3:627Ð637, 1964 60. Kepes JJ: Meningiomas. Biology, Pathology, and Differential 87. Newman SA, Jane JA: Meningiomas of the optic nerve, orbit, Diagnosis. New York: Masson, 1982, pp 3Ð5, 17Ð18, 119Ð120 and anterior visual pathways, in Al-Mefty O (ed): Menin- 61. Kim KS, Rogers LF, Lee C: The dural lucent sign: characteris- giomas. New York: Raven Press, 1991, pp 461Ð494

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88. Nicholson DH: Tumors of the optic nerve, in Smith JL (ed): 113. Spiller WG: Hemicraniosis and cure of brain by operation. Neuro-ophthalmology Update. New York: Masson, 1977, pp JAMA 49:2059Ð2065, 1907 131Ð143 114. Stern WE: Meningiomas in the cranio-orbital junction. J Neu- 89. Oka H, Kurata A, Kawano N, et al: Preoperative superselective rosurg 38:428Ð437, 1973 embolization of skull-base meningiomas: indications and lim- 115. Sternberg H: Ein Endothelium der Dura über einer innern itations. J Neurooncol 40:67Ð71, 1998 Exostose des Schadeldaches. Klin Wochenschr 56:178, 1919 90. Olivecrona H: Handbuch der Neurochirurgie. Berlin: 116. Subach BR, Lunsford LD, Kondziolka D, et al: Management Springer-Verlag, 1967 of petroclival meningiomas by stereotactic radiosurgery. Neu- 91. Pancoast HK, Pendergrass EP, Schaeffer JP: The Head and rosurgery 42:437Ð445, 1998 Neck in Roentgen Diagnosis. Springfield, IL: Charles C Tho- 117. Tattersall J: A case of cerebral tumor with tumor of the skull. mas, 1940 J Ment Sci 63:250Ð252, 1917 92. Penfield WG: Cranial and intracranial endotheliomata-hemi- 118. Teasdale E, Patterson J, McLellan D, et al: Subselective pre- craniosis. Surg Gynecol Obstet 36:657Ð674, 1923 operative embolization for meningiomas. A radiological and 93. Phemister DB: The nature of cranial hyperostosis overlying pathological assessment. J Neurosurg 60:506Ð511, 1984 endothelioma of the meninges. Arch Surg 6:554Ð572, 1923 119. Tedeschi F, Fragnito C, Brizzi R, et al: On the pathology of 94. Pieper DR, Al-Mefty O, Hanada Y, et al: Hyperostosis associ- meningiomas. A study of 412 cases. Acta Neuropathol Suppl ated with meningioma of the cranial base: secondary changes 7:119Ð121, 1981 or tumor invasion. Neurosurgery 44:742Ð747, 1999 120. Trobe JD, Glaser JS, Post JD: Meningiomas and aneurysms of 95. Pompili A, Derome PJ, Visot A, et al: Hyperostosing menigi- the cavernous sinus. Neuro-opthalmologic features. Arch omas of the sphenoid ridge—clinical features, surgical thera- Ophthalmol 96:457Ð467, 1978 py, and long-term observations: review of 49 cases. Surg Neu- 121. Uihlein A, Weyand RD: Meningiomas of anterior clinoid pro- rol 17:411Ð416, 1982 cess as a cause of unilateral loss of vision. Arch Ophthalmol 96. Probst C, Gessaga E, Leuenberger AE: Primary meningioma of 49:261Ð270, 1953 the optic nerve sheaths: case report. Ophthalmologica 190: 122. Vallat M, Robin A, Bokor J, et al: Meningiome du nerf optique 83Ð90, 1985 chez un enfant de deux ans. A propos d'un cas. Bull Soc Oph- 97. Quest DO: Meningiomas: an update. Neurosurgery 3: thalmol Fr 81:351Ð353, 1981 219Ð225, 1978 123. van Duyse D: Sarcome péridural avec invasion de l'espace 98. Rachlin JR, Rosenblum ML: Etiology and biology of menin- vaginal du nerf optique. Quelques considérations sur les tu- giomas, in Al-Mefty O (ed): Meningiomas. New York: Raven meurs intra- et extradurales et la métastase intravaginale éven- Press, 1991, pp 27Ð36 tuelle. Arch Ophthalmol 40:385Ð414, 1923 99. Reese AB: Tumors of the Eye, ed 3. Hagerstown, MD: Har- 124. Verheggen R, Markakis E, Muhlendyck H, et al: Symp- per & Row, 1976, pp 148Ð153 tomatology, surgical therapy and postoperative results of sphe- 100. Richter HP, Schachenmayr W: Preoperative embolization of noorbital, intraorbital-intracanalicular and optic sheath menin- intracranial meningiomas. Neurosurgery 13:261Ð268, 1983 giomas. Acta Neurochir Suppl 65:95Ð98, 1996 101. Rohringer M, Sutherland GR, Louw DF, et al: Incidence and 125. Virchow R: Entwickelungsgescichte des Krebes. Virchows clinicopathological features of meningioma. J Neurosurg 71: Arch 1:94, 1847 665Ð672, 1989 102. Rootman J: Disease of the Orbit: A Multidisciplinary Ap- 126. Walsh FB: Intracranial meningiomas: selected cases with neu- proach. Philadelphia: JB Lippincott, 1988, pp 354Ð357 ro-ophthalmologic interest, in Smith JL (ed): Neuro-ophthal- 103. Russell DS, Rubinstein LJ: Pathology of Tumours of the mology. Symposium of the University of Miami and the Nervous System, ed 5. London: E Arnold, 1989, pp 449Ð532 Bascom Palmer Eye Institute. Hallandale, FL: Huffman, 104. Salcman M: Malignant meningiomas, in Al-Mefty O (ed): 1970, Vol 5, pp 267Ð302 Meningiomas. New York: Raven Press, 1991, pp 75Ð85 127. Wara WM, Sheline GE, Newman H, et al: Radiation therapy 105. Sarkies NJ: Optic nerve sheath meningioma: diagnostic fea- of meningiomas. Am J Roentgenol Radium Ther Nucl Med tures and theraputic alternatives. Eye 1:597Ð602, 1987 123:453Ð458, 1975 106. Shapland CD, Greenfield JG: Case of neurofibromatosis with 128. Wilson WB: Meningiomas of the anterior visual system. Surv meningeal tumor involving left optic nerve. Trans Opthalmol Ophthalmol 26: 109Ð127, 1981 Soc UK 55:257Ð279, 1935 129. Wilson WB, Gordon M, Lehman RA: Meningiomas confined 107. Shuangshoti S: Meningioma of the optic nerve. Br J Oph- to the optic canal and foramina. Surg Neurol 12:21Ð28, 1979 thalmol 57:265Ð269, 1973 130. Wood MW, White RJ, Kernohan JW: One hundred intracra- 108. Sibony PA, Krauss HR, Kennerdell JS, et al: Optic nerve nial meningiomas found incidentally at necropsy. J Neuro- sheath meningiomas. Clinical manifestations. Ophthalmol- pathol Exp Neurol 16:337Ð340, 1957 ogy 91:1313Ð1326, 1984 131. Wright JE: Primary optic nerve meningiomas: clinical presen- 109. Smith JL, Vuksanovic MM, Yates BM, et al: Radiation thera- tation and management. Trans Am Acad Ophthalmol Oto- py for optic nerve meningiomas. J Clin Neuroophthalmol laryngol 83:617Ð625, 1977 1:85Ð99, 1981 110. Sood GC, Malik SK, Gupta DK, et al: Bilateral meningiomas of the orbit. Am J Ophthalmol 61:1533Ð1535, 1966 111. Sosman MC, Putnam TJ: Roentgenological aspects of brain Manuscript received April 10, 2001. tumors-meningiomas. AJR 13:1Ð12, 1925 Accepted in final form May 7, 2001. 112. Spiller WG: Cranial hyperostosis associated with underlying Address reprint requests to: Paul T. Boulos M.D., Box 800212 meningeal fibroblastoma. Arch Neurol Psychiatry 21: University of Virginia Health Sciences Center, Charlottesville, Vir- 637Ð640, 1929 ginia 22908. email: [email protected].

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