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Home , Diaphragma sellae, Falx cerebelli, Falx cerebri, Foster Kennedy syndrome, Pia mater, Sphenoid wing meningioma

CHAPTER 30 Tumors of the and Related Tissues: and Sarcomas

Kimberly P. Cockerham, John S. Kennerdell, Joseph C. Maroon, and Ghassan K. Bejjani

ANATOMY OFTHE MENINGES Associations Diagnosis Arachnoid Treatment Adjuvant Therapy MENINGIOMAS Clinical Characteristics by Location Histogenesis SARCOMAS OFTHE MENINGES AND Incidence Chondrosarcoma Pathology Osteogenic Sarcoma Cytogenetics Primary Sarcoma of the Meninges and Brain Endocrinology Rhabdomyosarcoma

ANATOMY OF THE MENINGES The meninges of the brain and consist of three into several freely communicating compartments. They in- different layers: the dura mater, arachnoid (tela arach- clude the , the tentorium cerebelli, the falx cere- noidea), and pia mater. Considerable anatomic differences belli, and the . exist among these structures, and these differences influence The falx cerebri, so named because of its sickle-like form, the nature, location, and spread of tumors that arise from is a fixed, arched process that descends vertically in the them. between the cerebral hemispheres (Fig. 30.2). The tentorium cerebelli is an arched lamina that is DURA MATER elevated in its midportion and inclines downward toward its peripheral attachments on both sides. It covers the superior The dura mater, typically referred to as the dura, is a thick surface of the and supports the occipital lobes membrane that is adjacent to the inner table of the and (Fig. 30.2). The is a small triangular process acts both as the functional of the skull and the of dura mater that lies beneath the tentorium cerebelli in outermost membrane of the brain (Fig. 30.1). In the spinal the posterior cerebellar notch between the two cerebellar canal, this dura is separated from bone by a space that con- hemispheres. It is attached superiorly to the undersurface of tains epidural adipose and areolar tissue. At the orbital end the posterior portion of the tentorium cerebelli. Its posterior of the optic canal, the dura encloses the intracanalicular por- and inferior attachments are to the and the tion of the and splits into an inner layer that . becomes the dural sheath surrounding the orbital portion of The diaphragma sellae is a small horizontal fold of dura the optic nerve and an outer layer that becomes the perios- that roofs the and almost completely covers teum of the walls of the , the periorbita. the (Fig. 30.3). The infundibulum extends In several areas of the brain, dural processes extend in- through an opening in the diaphragma that varies consider- ward. These processes or infoldings divide the ably in size (1,2). 1483 1484 CLINICAL NEURO-OPHTHALMOLOGY

Figure 30.1. The cerebral dura mater. The has been opened, together with its lateral lacunae, to expose arachnoid granulations. (From Gluhbegovic N, Williams TH. The : A Photographic Guide. Hagerstown, MD, Harper & Row, 1980.)

Figure 30.2. The falx cerebri (FC). This sickle- shaped dural structure is attached superiorly to the inner surface of the skull in the midline as far posteriorly as the internal occipital protuberance. This attachment contains the superior sagittal sinus (SSS). The inferior border of the falx cerebri arches over the (CC) and contains the inferior sagittal sinus (ISS). This border is at- tached to the (CG) anteriorly and the tentorium cerebelli (TC) posteriorly. (From Gluh- begovic N, Williams TH. The Human Brain: A Photographic Guide. Hagerstown, MD, Harper & Row, 1980.) TUMORS OFTHE MENINGES AND RELATED TISSUES 1485

Figure 30.3. The diaphragma sellae. A, Schematic drawing of the pituitary gland showing its relationship to the intracranial portion of the optic nerve (ON) and the optic chiasm (C). The gland is surrounded by dura except at the site of exit of the . The portion of dura covering the superior surface of the gland is the diaphragma sellae (arrowheads). B, Midsagittal section of the sellar region showing the pituitary gland and surrounding structures. The diaphragma sellae covers the entire superior surface of the gland except in the region of the stalk. (From Renn WH, Rhoton AL Jr. Microsurgical anatomy of the sellar region. J Neurosurg 1975;43Ϻ288–298.)

ARACHNOID The arachnoid is a delicate membrane that consists of bundles of white fibrous and elastic tissue intimately blended together (Figs. 30.4 and 30.5). Its outer surface is covered with at least one, and often as many as ten, layers of flat, elongated cells (arachnoid cap cells). These cells are sepa- rated from the rest of the arachnoid layer by a thin basal lamina. In the region of the arachnoid villi, they serve as conduits for the (CSF) into the venous circulation of the dural sinuses.

Figure 30.4. The cerebral arachnoid. On the superolateral surfaces of both cerebral hemispheres, the arachnoid appears as a delicate, transparent membrane through which the contours of the cerebral gyri can be seen, along with the venous and arterial channels that traverse the subarachnoid Figure 30.5. Schematic diagram of a coronal section of the meninges and space. Note the clusters of arachnoid granulations along the margins of the the , showing the relation of the dura mater, arachnoid, and longitudinal cerebral fissure. (From Gluhbegovic N, Williams TH. The pia mater. Note the presence of an arachnoid villus and its relationship Human Brain: A Photographic Guide. Hagerstown, MD: Harper & Row, to a dural venous sinus. (From Weed LH. The formation of the cranial 1980.) subarachnoid spaces. Am J Anat 1923;31Ϻ191–221.) 1486 CLINICAL NEURO-OPHTHALMOLOGY

The arachnoid is tightly adherent to the dura so that there of age and increase in number throughout life. Large granu- is only a potential space between them, the . lations are called pacchionian bodies. The arachnoid villi The cap cells that form the outer barrier layer of the arach- and granulations are responsible for transport of CSFfrom noid and the border cells of the inner layer of the dura are the subarachnoid space to the cerebral dural sinuses. tightly fused together, forming the interface layer (3–5). In contrast, the arachnoid is separated from the pia mater by PIA MATER the CSF-filled subarachnoid space. Although the arachnoid The innermost of the three layers of the meninges, the pia is rather closely apposed to the pia mater on the surface of mater, consists of a delicate, flat sheet of cells that is re- the brain, it does not extend into cerebral or cerebellar sulci. flected from the surface of the brain to form the outer coating The arachnoid villi invaginate the dura to project into the of the meningeal vessels in the subarachnoid space (Fig. venous sinuses (Fig. 30.5). Collections of these villi, forming 30.5). The pia mater of the brain is generally thinner than cauliflower-like clumps, are called arachnoid granulations that of the spinal cord. In most areas, it is connected with (6) (Fig. 30.1). They begin to develop at about 18 months the arachnoid by fine trabeculae lined by mesothelial cells.

MENINGIOMAS In the past, these tumors were called ‘‘dural endothelio- Intracranial meningiomas are most commonly detected in mas,’’ ‘‘arachnoid fibroblastomas,’’ or ‘‘meningeal fibro- the middle and late decades of life (18). Meningiomas ac- blastomas.’’ These terms implied an understanding of the count for only 2–4% of primary intracranial tumors in pa- basic histogenesis of the tumor; however, it eventually be- tients less than 20 years of age (22–24). came evident that the embryologic derivation of the tumor Some of the meningiomas that occur in childhood appear cells was far from clear. Accordingly, Cushing introduced to be congenital, producing symptoms in the first few months the term ‘‘’’ to identify tumors that originate of life (25–28). These tumors tend to be of the convexity from cells of the meninges but without specifying their pre- type and are often associated with large peritumoral cysts. cise histogenesis (7–12). Most meningiomas in children, however, occur later, in the HISTOGENESIS first and second decades of life. Neurofibromatosis and pre- vious head irradiation are both significant risk factors. One Meningiomas may be attached to any of the three layers quarter of childhood meningiomas are associated with neu- of the meninges. They originate from the arachnoid cap cells rofibromatosis. In a large series of children who had received that line the outer surface of the arachnoid and are fused with the dural border cells to produce the interface layer radiation for tinea capitis, the relative risk of developing an between the dura and the arachnoid. Although some authors intracranial meningioma was increased fourfold (24). believe that these cells are derived from the and Meningiomas that occur in childhood tend to be more are thus of neuroectodermal origin, it appears more likely aggressive than their adult counterparts (21,22). In addition, that they are of mesenchymal origin (13,14). Nevertheless, malignant degeneration, often associated with necrosis, oc- the possibility remains that neural crest elements contribute curs in 50% of pediatric meningiomas (24). There are, never- to the development of the meninges (11,15). theless, many cases of benign childhood meningiomas Cap cells are not confined to the outer layer of the arach- (29,30). Both cyst formation and intraventricular location are noid. They are also found in the stroma of the normal more characteristic of meningiomas presenting in childhood plexus, particularly in the heavy tufts of the glomus portion (24,31,32). In contrast, symptomatic intracranial meningio- in the region of the trigone of the . These mas are not uncommon in older patients (18,21). cells are never located on the ventricular surface but are Meningiomas occur about two to three times more fre- separated from it by the epithelium of the . quently in women than in men. The reason for this female The presence of arachnoid cell clusters in this location may predominance is not clear but may be related to estrogen be explained by the invagination of the leptomeninges that and progesterone receptors in these tumors. Interestingly, forms part of the . The arachnoid cap cells in the gender difference does not occur in children with menin- the stroma of the choroid plexus of the lateral, third, and giomas (8,11,33). fourth ventricles are the source of intraventricular meningi- It has long been recognized that patients with neurofibro- omas. matosis, a familial disease inherited as an autosomal-domi- INCIDENCE nant trait (see Chapter 38), are susceptible to meningiomas, Intracranial meningiomas represent approximately 20% often multiple ones (8,34). There have, however, been re- of all primary intracranial tumors in most large series ports of meningiomas occurring in several members of fami- (8,16–18). The age-adjusted incidence is 0.9 and 1.92/ lies without evidence of neurofibromatosis (35–37). The 100,000 for men and women, respectively, with an annual presence of more than one meningioma in a single patient incidence of 8/100,000 in more recent studies (19,20). Me- is not rare and has been known since the beginning of the ningiomas in the are far less common and ac- 20th century (8,9,38–47). Cushing and Eisenhardt subdi- count for less than 10% of all meningiomas. There is a dis- vided multiple meningiomas into three groups: those occur- tinct predilection that varies only slightly by location ring in patients with neurofibromatosis; those occurring in (7,19,21). patients without neurofibromatosis; and meningiomatosis TUMORS OFTHE MENINGES AND RELATED TISSUES 1487

(numerous ‘‘tumors’’ of various sizes found studding the age. With the advent of advanced neuroimaging, many inner surface of the dura mater) (8). asymptomatic meningiomas are identified in persons being The incidence of multiple meningiomas was believed to studied for other reasons (51,52). be 1–2% before the advent of gadolinium-enhanced mag- netic resonance (MR) imaging but is now recognized to be PATHOLOGY at least 10% (10,48,49). Meningiomas range in size from tiny to massive. The Meningiomas commonly remain asymptomatic through- characteristic gross appearance is a globular, solid growth out life and may be discovered only during autopsy (39,50). firmly attached to the inner surface of the dura, producing The prevalence of incidental meningiomas increases with an excavation of the underlying neural tissue (Fig. 30.6).

Figure 30.6. Relationship of meningiomas to adjacent brain. A, Large parasagittal meningioma has compressed the right frontal lobe and has displaced the frontal portion of the left hemisphere away from the midline. The tumor is clearly separate from the surface of the brain. B, This small meningioma of the right frontal region was an incidental finding at autopsy. It is attached not to the brain but to the dura. The tumor has compressed and excavated but not invaded the portion of the brain adjacent to it. C, Coronal section through the brain of a patient with a sphenoid ridge meningioma. The tumor clearly compresses but does not invade the right frontal and temporal lobes. (B, From Rubinstein LJ. Tumors of the . 2nd series, 6th fascicle. Washington, DC: Armed Forces Institute of Pathology, 1972.) 1488 CLINICAL NEURO-OPHTHALMOLOGY

membranes, but these membranes are extensively inter- twined, resulting in the appearance of a syncytium. Transitional meningiomas have two distinctive cell popu- lations: the plump, polygonal cells observed in syncytial me- ningiomas and the spindle-shaped cells tightly wrapped around each other to form a whorl (Fig. 30.7). In tumors with extensive whorl formation, psammoma bodies are usually present (Fig. 30.8). Transitional meningiomas that contain extensive psammoma bodies are also called psammomatous meningiomas. Fibroblastic meningiomas are composed of interlacing bundles of spindle-shaped cells containing conspicuous fi- broglial fibrils (Fig. 30.9). The cell nuclei are narrow and rod-shaped. These tumors contain some whorls, although they are less obvious and more poorly formed than those observed in transitional meningiomas. Psammoma bodies are occasionally present. A distinctive feature of fibroblastic meningiomas is the extensive development of reticulin and collagen fibers between individual cells, producing a tough, ‘‘fibrous’’ structure. Most intraventricular and spinal cord meningiomas are of the fibroblastic type. Angioblastic meningiomas are characterized by extensive blood vessels that resemble in size and structure (Fig. 30.10). Some lumina are dilated and sinusoidal, whereas endothelial cells occlude others. Mitotic figures are Figure 30.7. Histopathologic appearance of a transitional meningioma. not uncommon. These cells do not form the cellular whorls Conspicuous whorl formations of elongated and crescent-shaped meningi- characteristic of transitional (and to a lesser extent fibroblas- oma cells are present. (From Rubinstein LJ. Tumors of the Central Nervous tic) meningiomas. The two most common variants of angio- System. 2nd series, 6th fascicle. Washington, DC: Armed Forces Institute of Pathology, 1972.)

The cut surface of a meningioma may have a reddish or brown discoloration from hemorrhage within the tumor. In some cases, the tumor contains one or more large cysts, whereas in extremely rare cases, the tumor is totally cystic (8,53–58). In other cases, particularly in infants and young children, there is a large cyst located adjacent to the tumor (27,56,58,59). Tumors with extensive hemorrhage, cyst for- mation, or advanced mucoid or fatty degeneration usually have a soft, spongy consistency. Other tumors, however, have a tough, fibrous consistency. Still others contain exten- sive areas of calcification and are ‘‘gritty’’ to palpation. Meningiomas have a variable degree of vascularity. Highly vascularized tumors may bleed extensively from fresh-cut surfaces, whereas less vascularized tumors bleed almost not at all. A macroscopic variant of the typical meningioma is the meningioma-en-plaque, which, as its name suggests, is not greatly raised above the level of the dura mater but invades the adjacent bone without producing hyperostosis initially. There are five major microscopic subtypes of meningio- mas: syncytial, transitional, fibroblastic, angioblastic, and malignant (60). The clinical importance of these subdivi- sions is debated. Syncytial meningiomas lack a boundary between neigh- Figure 30.8. Numerous psammoma bodies are present in a transitional boring cells when examined under the light microscope. meningioma. (From Rubinstein LJ. Tumors of the Central Nervous System. Electron microscopy, however, shows that the tumor is not 2nd series, 6th fascicle. Washington, DC: Armed Forces Institute of Pathol- a true syncytium. The tumor cells do possess individual cell ogy, 1972.) TUMORS OFTHE MENINGES AND RELATED TISSUES 1489

Figure 30.9. Histopathology of fibroblastic meningioma. The highly elongated meningioma cells resemble fibroblasts and form closely interlac- ing bundles. (From Rubinstein LJ. Tumors of the Central Nervous System. 2nd series, 6th fascicle. Washington, DC: Armed Forces Institute of Pathol- ogy, 1972.) Figure 30.10. Histopathology of angioblastic meningioma. The tumor cells lie adjacent to numerous, thin-walled capillaries. (From Rubinstein LJ. Tumors of the Central Nervous System. 2nd series, 6th fascicle. Wash- blastic meningiomas are the hemangioblastic and hemangio- ington, DC: Armed Forces Institute of Pathology, 1972.) pericytic types (61,62). We agree with those who believe that there is a separate tumor called a hemangiopericytoma that is composed of pericytes and is embryologically, clini- The clinically benign meningioma merely compresses but cally, and morphologically different from the angioblastic does not infiltrate the CNS, regardless of how extensively meningioma. it encroaches upon neighboring tissues. When there is histo- The criteria for malignancy in meningiomas are confus- logic evidence of local invasion, the tumor is considered ing. These tumors are rarely classified as malignant meningi- ‘‘malignant.’’ This type of appearance is most frequently omas on histologic grounds alone (10,63–65). Clinically ag- observed in the angioblastic meningioma (21,68,78,79). gressive meningiomas tend to have a papillary configuration, Meningiomas with no detectable histologic features of increased cellularity, and areas of focal necrosis. The cells malignancy nevertheless occasionally metastasize outside show pleomorphism and numerous mitoses (Fig. 30.11). and within the CNS (67,80,81). Cushing and Eisenhardt Many but not all of these tumors have angioblastic features. mentioned pulmonary metastases, and other sites of metasta- These tumors tend to invade adjacent cerebral tissue and to ses include the bones, liver, and lymph nodes (8,10,73–77, excite proliferative changes that exceed those of ordinary 82,83). gliosis in the neighboring glial cells (10,63–68). As might Dissemination of meningioma along the CSFpathway is be expected, meningiomas that are histologically malignant even less common than is distant metastasis (21,84). Most recur more often and sooner after surgery than do meningio- often, dissemination is associated with histologic features of mas that are histologically benign, even when postoperative malignancy in a previously resected primary tumor (21,68). radiotherapy is used (69,70). In addition, such tumors have a tendency to metastasize both within and outside the central CYTOGENETICS nervous system (CNS) (65,71–77). However, histologically ‘‘benign’’ meningiomas may The desire to differentiate benign from malignant menin- also grow rapidly, become locally invasive, and even metas- giomas and to predict which tumors have a greater propens- tasize. Thus, the criteria for malignancy are more commonly ity to recur has prompted new methods of tumor analysis. based on their biologic behavior (67). The bromodeoxyuridine (BUdR) labeling index can be used 1490 CLINICAL NEURO-OPHTHALMOLOGY

mas and are thought to actively induce tumor formation. Other secondary aberrations, such as deletions, have been reported (42). Certain deletions (1p, 6q, 10p, 10q, 14q, and 18q) have been associated with higher-grade meningiomas and with progression or recurrence. The details of the roles of suppressor and induction genes in meningioma development remain to be defined (9,10,42–46,70,89). ENDOCRINOLOGY In 1929, Cushing and Eisenhardt observed that meningio- mas, which occur much more frequently in women than in men, may become symptomatic during pregnancy (90). It has subsequently become clear that meningiomas frequently enlarge or become symptomatic during pregnancy and dur- ing the luteal phase of the menstrual cycle (91,92). There is no evidence, however, that meningiomas present more often during pregnancy (93,94). In addition, there is an increased incidence of meningioma among women with breast carci- noma—the only significant association between a nervous system neoplasm and a primary malignancy elsewhere in the body (95–98). These findings suggest that at least some meningiomas are under hormonal influence. There is extensive biochemical evidence that some menin- giomas contain both estrogen and progesterone receptors; Figure 30.11. Histopathology of malignant meningioma. The cells are however, the presence of progesterone receptors, even in a somewhat pleomorphic, and mitoses are present. small number of tumor cells, has been reported to be a favor- able prognostic factor, and such receptors may be present in normal human leptomeninges from which these tumors to predict aggressive tumor behavior. Hoshino et al. applied arise (99–125). Although the percentage of meningiomas these techniques and were able to demonstrate that the me- with estrogen and progesterone receptors varies consider- ningiomas in six of nine patients with a BUdR index greater ably among studies (possibly relating to the use of preopera- than 1% were more aggressive and likely to recur (85). All tive glucocorticoid therapy), it is clear that such recep- meningiomas with a BUdR index higher than 5% recurred. tors are present in a substantial number of meningiomas Using a different approach, Chin and Hinton identified a (108,120). In contrast to breast cancer, progesterone receptor significant difference between the number of argyrophilic positivity is more common (72–84%) than estrogen receptor nucleolar organizer regions (Ag NOR) in benign, atypical, positivity (31–33%) (122,126). In vitro, in vivo, and clinical and malignant meningiomas and were able to identify tumors studies support the notion that the progesterone receptors are with a greater likelihood for recurrence (86). functional. Rubinstein et al. evaluated primary and recurrent Flow cytometry techniques have demonstrated that recur- intracranial meningiomas from 51 patients for the presence rent meningiomas have a higher proliferative index, and tu- of receptors for progesterone and estrogen (122). They found mors with a proliferative index greater than 20% recurred that the high progesterone positivity (84%) in primary tu- independently of histopathologic characteristics (87). mors was even more prevalent in recurrent meningiomas Hepatic growth factor (HGF) is a cytokine that promotes (92%), independent of prior corticosteroid treatment (124). cell growth and angiogenesis by binding to the tyrosine- Anti-estrogen agents are unsatisfactory in the treatment kinase receptor, encoded by the photo-oncogene c-Met. Ar- of meningiomas, and the clinical experience with anti-pro- rieta et al. found in 11 patients that high levels of HGF gesterone therapy (mifepristone) is still limited (126,127). correlated with meningioma recurrence, vasogenic edema, Anti-progesterone side effects, although typically mild, in- proliferation index, and vascular density (88). clude cessation of menses, hot flashes, gynecomastia, partial Chromosomal analysis provides an improved understand- alopecia, and fatigue (126,128). ing of the genetic predisposition for meningioma formation. Receptors other than estrogen or progesterone are also Patients with meningiomas, type 2 neurofibromatosis, and present in meningiomas. Using the polymerase chain reac- breast cancer all share a deletion of the tumor suppressor tion, mRNA for dopamine and prolactin receptors were iden- gene on the long arm of chromosome 22. Seventy-two per- tified in meningioma tissue specimens and cell cultures de- cent of patients with meningiomas have monosomy of chro- rived from meningioma tissue (129). This finding might mosome 22, whereas a hyperdiploid state may accompany explain prior observations that dopamine, bromocriptine, more malignant tumors. One copy of the gene is lost during and selective D1 agonists decrease meningioma cell culture development, and radiation, viruses, or trauma may alter a proliferation (130). In contrast to tumors with high prolifera- second copy. In addition, oncogenes (HA ras, C-fos, Myc, tion rates (e.g., glioblastomas), benign meningiomas do not C-erb B) have been identified in association with meningio- have detectable telomerase activity (42,131,132). TUMORS OFTHE MENINGES AND RELATED TISSUES 1491

ASSOCIATIONS raphy may be required preoperatively, especially if preopera- tive embolization is considered (150,151). Patients with meningiomas may harbor other intracranial Gadolinium-enhanced MR imaging also plays an impor- or intraspinal tumors. A combination is most often seen in tant role in postoperative surveillance for progression and patients with neurofibromatosis type 1 (NF-1), in whom me- recurrence. Persistent postoperative inflammatory changes ningiomas may be associated with acoustic neuromas, neuro- can create occasional diagnostic dilemmas, but the sensitiv- fibromas, and gliomas. In addition, individual patients with- ity of MR imaging in detecting early tumor change is supe- out neurofibromatosis may have meningiomas and gliomas, rior to that of CT scanning (10,152–163). pituitary adenomas, and other less common tumors (11,133,134). Russell and Rubinstein believed that most of TREATMENT these cases represent the coincidental occurrence of two un- related tumors in the same patient, and we agree that in most Surgical excision remains the mainstay of treatment for instances this seems to be the case (21). meningiomas. Unfortunately, tumor location and proximity Metastasis of carcinoma to benign and other malignant to vital neural structures (e.g., ) often limits tumors occurs infrequently. Meningiomas, however, are the tumor resection. In patients with subtotally resected tumors, host tumor in 80% of the benign tumors reported (135–137). radiation therapy remains the preferred first line in adjunc- tive therapy. In contrast to the hormonal therapies, the chem- otherapeutic agent hydroxyurea has been used with some DIAGNOSIS success in clinically malignant tumors. Symptoms and signs vary by location, but epilepsy is a Meningiomas can be very well vascularized, so preopera- common presenting symptom in patients with meningiomas. tive embolization is often required to enhance surgical exci- Of 22 patients with surgically treated meningiomas, 27% sion. Endovascular therapy has evolved over the past decade, suffered from epilepsy (138). allowing selective and safe embolization. The timing of em- Convexity and parasagittal/falx locations were most com- bolization remains controversial. Kai et al. suggested that mon. Tuberculum sella, sphenoid ridge, and olfactory groove the interval between embolization and surgery should be 7 locations have been reported. Peritumoral edema was a sig- to 9 days in meningiomas receiving more than 50% of their nificant factor regardless of location. Generalized seizures supply from the external carotid artery (164–166). Sequen- are the most prevalent. tial MR imaging and MR spectroscopy have been used to High-resolution computed tomography (CT) scanning and assess revascularization and fatty degeneration in meningio- gadolinium-enhanced MR imaging have revolutionized the mas that are embolized but where surgery has been delayed diagnosis of meningiomas (139–145). CT scanners ade- (160). Challenges remain, including feeding branches that quately sensitive to identify hyperostosis and calcification are difficult to access with even the most advanced superse- became available in the early 1980s, but gadolinium-DPTA lective microcatheters (166). was not widely used until around 1985. Prior to the availabil- Technology continues to enhance operative capabilities. ity of this paramagnetic contrast agent, MR imaging was Specialized all-angle neurosurgical operating microscopes, limited by the fact that most meningiomas appeared isoin- self-retaining retractor systems, medical lasers, and a myriad tense to brain. On neuroimaging, meningiomas are typically of intricate instruments allow reduction of postoperative smooth, homogenous, round or oval masses. Heterogeneity morbidity and mortality (9,10). Sophisticated electrophysio- or mottling, suggestive of an angioblastic or malignant sub- logic intraoperative monitoring devices are now routinely type, occasionally occurs. On T1-weighted images, 60–90% used in skull base surgery when the proximity to cranial of meningiomas are isointense to gray matter; the remainders nerves enhances the surgical challenge. Continuous monitor- are slightly hypointense. On T2-weighted images, meningio- ing expedites identification and dissection of the cranial mas may be slightly hyperintense (30–45%) or isointense nerves (II–XII) that are often structurally altered by the (50%). A clear peritumoral rim is seen in 50% of intracranial tumor. meningiomas, appearing hypointense on T1-weighted im- Frameless stereotaxy integrates preoperative imaging ages and hypo- or hyperintense on T2-weighted images. with intraoperative anatomy. Immediate feedback on the lo- Most diagnostic, however, is the marked, homogenous en- cation and the relation of the tumor to distorted anatomy is hancement that occurs with gadolinium-DPTA and is inde- provided (167–173). pendent of tumor location. A thickened tail of dural enhance- Results of microsurgical restoration of function of cranial ment is commonly seen extending away from the mass edge; nerves are improving. Now, when vital structures are inti- this is particularly true of falx and tentorial meningiomas. mately involved or inadvertently injured, new techniques are This finding is characteristic but not pathognomonic of me- available to repair and to graft the internal ningiomas (146,147). Edema of the white matter in the re- carotid artery. gion surrounding the tumor is best demonstrated on T2- ADJUVANT THERAPY weighted scans. The edema can also be visualized with CT scanning, but MR imaging is superior to CT scanning at Recurrence (or progressive growth of remaining tumor) defining vascular encasement and tumor vascularity (148). is a problematic feature of meningiomas (88,174,175). The The information obtained from the flow voids is increased key to preventing recurrence of intracranial meningiomas is when MR angiography is performed (149). Standard angiog- complete surgical excision (176–179). However, indepen- 1492 CLINICAL NEURO-OPHTHALMOLOGY dent risk factors include radiation-induced tumors (espe- Table 30.1 cially if the radiation was delivered during childhood) and Novel Treatments for Recurrent Meningiomas chromosomal alteration. Ja¨a¨skela¨inen evaluated the rate of late recurrence and factors predicting recurrence in 657 pa- Angiogenesis inhibition tients (66) and found a recurrence rate at 20 years of 19% Interferon-alpha TNP-470 (synthetic analogy of fumagillin) using life-table analysis. In an attempt to decrease recurrence Tumor necrosis factor rates when complete excision is difficult (e.g., medial sphe- PD156707 noid wing, cavernous sinus, and olfactory groove meningio- Verotoxin mas), adjuvant therapy can be used. A wide variety of mark- Inhibition of tumor cell growth ers have been reported to help predict which meningiomas Interferon-alpha will recur, but at this time none are used clinically in most Hydroxyurea patients with meningiomas. Growth hormone blockade Radiation therapy is the only validated adjuvant therapy Pegvisomant (181). Stereotactic is used with some success, Octreotide but postoperative external beam radiation has more wide- Bromocriptine spread application (182–185). Radiation therapy should be Growth factor blockade delayed at least 2 months postoperatively and then carefully Trapidil fractionated (200 cGy/day) for a total dose of 5,000–5,400 Suramin Bromocriptine cGy to minimize potential complications (186). Treatment Signal pathway inhibition by a qualified radiation oncologist is routinely considered Calcium channel blockers when surgical resection is incomplete or the tumor is recur- Mitogen-activated protein kinases rent (187,188). Potential complications (, optic Gene therapy neuropathy, cerebral necrosis, and secondary malignancies) Herpes simplex or adenovirus transduction for merlin gene are minimized with correct dosimetry, fractionation, and dosing (183–185,189–191). Whether intensity-modulated Adapted from Ragel B, Jensen RL. New approaches for the treat- x-rays or proton beams are used, stereotactic therapy allows ment of refractory meningiomas. Cancer Control 2003;10 : 153. a more focused and therefore safer delivery system (dosimet- ric optimization) (192–194). Radiation remains stigmatized by the occasional complications that are more prevalent wide variety of other options will be explored in the next when the therapy is delivered by the inexperienced or deliv- decade (Table 30.1). ered without fractionation (gamma knife) (195–200). Highly conformal therapy with protons may prove to be safer, but CLINICAL CHARACTERISTICS BY LOCATION a large prospective case series comparing immunomodulated radiation therapy to conformal therapy with protons would The location of a meningioma determines its clinical pre- need to be conducted (185,193,194). sentation. Familiarity with presenting The clinical experience with microsurgical implantation is important, not only because they are often of neuro-oph- of radioactive seeds, brachytherapy, is limited due to con- thalmologic interest, but also because meningiomas are gen- cerns about seed migration and potential damage to adjacent erally benign and thus can often be successfully treated if neurovascular structures (201,202). the diagnosis is made early. The specific sites of involvement Standard chemotherapeutic agents can be used but have are discussed in alphabetical order. minimal effect in the malignant forms of meningioma (10). Trapidil, a platelet-derived growth factor agonist, has a sig- Cavernous Sinus Meningiomas nificant effect in vitro but has not been used in patients or experimental animal models. Minimizing recurrence rates, These tumors probably do not arise within the cavernous especially in patients with malignant meningiomas, remains sinus, but rather from the meninges covering the floor of the a challenge (180,203). Hydroxyurea is a chemotherapeutic middle fossa in the region of the petrous apex. Meningiomas agent (20 mg/kg/day) that is administered orally. It has been that involve the cavernous sinus usually produce slowly pro- used with some success in patients with unresectable or re- gressive symptoms and signs related to damage to the ves- current meningiomas that are histologically benign. The re- sels, cranial nerves, and sympathetic plexus within the sinus ported response rate with stabilization of the neuroradiologic (207–213). appearance varies from 75% to 88%. Hydroxyurea is usually , the most common symptom, results from paresis well tolerated, but hematologic toxicity, especially leukope- of one or more of the ocular motor nerves. Damage to the nia, may occur, requiring reduction in daily doses or drug within the cavernous sinus may produce a discontinuation (204,205). variety of clinical syndromes, including a partial or complete Verotoxin 1 (VT1) is an E. coli toxin that targets globotri- -sparing that initially may be aosylceramide (Gb3) glycolipid on tumor cells acting with mistaken for the produced by myasthenia antineoplastic action. Gb3 expression is much more common gravis, an oculomotor nerve palsy with the pupil involved, in malignant (82%) meningiomas than the histologically be- or an oculomotor nerve palsy with the pupil smaller than nign variant (20%). Animal research has been promising, normal from damage to the oculosympathetic pathway (207) but clinical application is an essential next step (206). A (Figs. 30.12 and 30.13). In addition, involvement of the ocu- TUMORS OFTHE MENINGES AND RELATED TISSUES 1493

Figure 30.12. Oculomotor nerve palsy associated with a pupil that is smaller than the contralateral pupil because of involvement of the oculosympathetic pathway in the cavernous sinus. The patient was thought to have a basal meningioma on the basis of neuroradiologic studies. A, The patient has a complete of the right upper . B, When the right upper eyelid is elevated manually, the right eye can be seen to be proptotic and exotropic. The right pupil is slightly smaller than the left pupil. The right eye cannot adduct (C) or elevate (D), although it can depress slightly (E). 1494 CLINICAL NEURO-OPHTHALMOLOGY

Figure 30.13. Proptosis and incomplete oculomotor nerve palsy with in- volvement of the oculosympathetic pathway in a 62-year-old woman with a presumed meningioma in the left cavernous sinus. A, There is left proptosis and mild . The left pupil is slightly smaller than the right, although there is no ptosis apparent. The left pupil did not dilate after topical instilla- tion of 10% cocaine. B–E, There is full abduction of the left eye, but adduction, elevation, and depression of that eye are limited. lomotor nerve may cause primary aberrant regeneration trochlear nerve paresis occurs in association with oculomo- (207,213–215) (Fig. 30.14). Patients with this syndrome tor or abducens nerve paresis. In addition, abducens nerve have evidence of aberrant regeneration of the oculomotor paresis is often associated with other ocular motor nerve nerve but no history of acute oculomotor nerve palsy. Aneu- dysfunction with and without facial pain (207,216–218). In rysms and other slowly growing lesions within the cavernous some instances, damage to the abducens nerve within the sinus and in the subarachnoid space also may produce the cavernous sinus also results in damage to the oculosympa- syndrome of primary aberrant regeneration. In contrast, thetic pathway, because the latter runs for a short distance TUMORS OFTHE MENINGES AND RELATED TISSUES 1495

with the abducens nerve in the cavernous sinus before join- ing the ophthalmic division of the (219,220). This produces a unilateral abducens nerve paresis associated with an ipsilateral postganglionic Horner’s syn- drome (Fig. 30.15). If the trigeminal nerve is involved, pa- tients may complain of dysesthesia or, rarely, pain in and around the eye, the orbit, or the upper face (212,213,218, 221,222). These symptoms are a result of trigeminal nerve involvement. The optic nerve may be damaged by growth of the tumor through the superior orbital fissure into the orbit or by exten- sion of the tumor to involve the intracranial or intracanalicu- lar portions of the nerve (210). When cavernous sinus menin- giomas compress the venous structures within that region or extend into the orbit, mild proptosis may occur (207,208,223). Gadolinium-enhanced MR imaging (with specific atten- tion to the cavernous sinus) demonstrates early and subtle loss of the normal convexity of the lateral wall of the caver- nous sinus, an increased density within or adjacent to the sinus, an asymmetry in the degree of enhancement, or a combination of these features (213,224–230). When angiography is performed, cavernous sinus menin- giomas characteristically demonstrate abnormal vessels aris- ing from both the internal and the external carotid arteries supplying the tumor. An enlarged meningohypophyseal trunk and an enlarged inferior cavernous sinus artery, both of which arise from the intracavernous portion of the internal carotid artery, are the abnormalities most commonly encoun- tered. Invasion of the internal carotid artery has been de- scribed (231). Tumor vessels originating from the external carotid artery via the middle meningeal artery, accessory meningeal artery, and artery of the foramen rotundum may also be seen, particularly when the meningioma is large. Figure 30.14. Primary aberrant regeneration in a 45-year-old woman with Most meningiomas produce a classic, homogeneous tumor a left intracavernous meningioma. A, In primary position, the patient fixes ‘‘blush’’ that may be seen during the and venous with her right eye. She has a slight left ptosis, an exotropia, and phases of the angiogram (208,209). Angiography is particu- with the larger pupil on the left. B, On attempted gaze down and right, the larly important when hyperostosis is extensive and surgical left upper eyelid elevates and the left pupil constricts. excision is planned. Preoperative embolization should be strongly considered. The treatment of meningiomas that involve the cavernous sinus is in evolution. If symptoms and signs are progressive

Figure 30.15. Left abducens nerve paresis associated with left postganglionic Horner’s syndrome in a 65-year-old man with a presumed left intracavernous meningioma. Note the mild left ptosis and . 1496 CLINICAL NEURO-OPHTHALMOLOGY or significant growth is demonstrated on serial imaging stud- it does appear to do so in some (208,241–244). Alterna- ies, intervention is warranted. Total removal of the mass tively, with stereotactic radiosurgery, high-energy radiation without damaging cranial nerves or the internal carotid is may arrest the growth of some tumors (245). Many centers often difficult, but with microsurgical techniques, it is possi- routinely use external beam radiation, or fractionated stereo- ble to operate within the cavernous sinus with a relative tactic radiation therapy, for cavernous sinus meningiomas degree of safety (232). A variety of approaches are used, either primarily or after confirmation of diagnosis and subto- including subtemporal, pterional, orbito-zygomatico-malar, tal resection of tumor (188,232,246,247). and transsphenoidal. The procedure is dictated by the charac- Some investigators recommend that meningiomas that teristics of the tumor and the preference of the surgeon cannot be totally removed, such as those within the caver- (212,228–237). Rare meningiomas, such as those attached nous sinus, should be biopsied and the specimens assayed to the outer wall of the cavernous sinus, may be completely for the presence of estrogen and progesterone receptors. The removed. Those within the cavernous sinus can be signifi- role of a nonsteroidal anti-estrogen, such as tamoxifen cit- cantly reduced in size, but there is significant risk to the rate, is controversial (127,248,249). Other agents have also oculomotor, trochlear, and abducens nerves if total removal been tried, such as the antiprogesterone agent mifepristone, is attempted. Proximal and distal control of the internal ca- with mixed success (126). Even if the therapy is successful, rotid artery is essential. If the artery is encased with tumor, lifelong therapy is required. cerebral revascularization (with a saphenous vein graft or When the signs and symptoms are minimal, meningiomas external carotid anastomosis) may be necessary (238,239). in this location may be followed, because tumor growth is If neuroimaging is suggestive of encasement, a preoperative often slow (210,250). Indeed, some patients with these tu- balloon occlusion test of the internal carotid artery is per- mors experience spontaneous regression of symptoms and formed to evaluate the effect on cerebral blood flow. Preop- signs (210,251). The prognosis for binocular single vision erative MR imaging and angiography are very helpful in in patients with meningiomas of the cavernous sinus is not characterizing the relationship to the carotid and the location particularly good, but prognosis for life is excellent, regard- of the tumor (sellar, sphenoid sinus, orbital apex). Hirsch et al. found this information predictive of the difficulty of less of treatment (237). This should be kept in mind when resection and likelihood of postoperative complications considering the form of treatment to be used, particularly in (229). As revascularization techniques have improved, nerve older patients. grafting has also evolved. Cranial nerves have been success- fully resutured directly or with an interposition graft. Sekhar Convexity Meningiomas et al. performed direct end-to-end anastomosis in 5 nerves and 11 interposition nerve grafts and observed improvement The most frequent site of meningiomas is the cerebral or recovery of cranial nerve function (III, IV, V, or VI) in convexity (178). Convexity meningiomas originate from the 13 of 16 nerves (240). The oculomotor nerve had the most meninges of the cranial vault in locations other than the variable results because of the potential for aberrant regener- base of the skull, falx, or sagittal sinus (Fig. 30.16). Some ation. convexity meningiomas enlarge until they abut the superior A good alternative to aggressive surgical resection is ex- sagittal sinus. When this occurs, they are considered parasag- ternal beam radiation to the region of the tumor. Tumor ittal meningiomas. In most cases, convexity meningiomas debulking or no surgery can precede. Although such therapy are located in the frontal and parietal regions, rather than in does not reduce the size or rate of growth in all meningiomas, the temporal or occipital regions. More than 70% of convex-

Figure 30.16. Asymptomatic convexity meningi- oma found at autopsy in a 76-year-old woman. Note compression without invasion of adjacent brain. TUMORS OFTHE MENINGES AND RELATED TISSUES 1497 ity meningiomas are frontal and located anterior to the sulcus embolization in selected tumors (256–258). According to (12). most authors, meningiomas may be separated into four an- Seizures are a common early symptom of frontal, parietal, giographic types: those with a pure external carotid artery and temporal meningiomas. The seizures may be either focal supply; those with a mixed external/internal carotid artery or generalized (252). In rare instances, a seizure may be supply but with dominant feeders from the external carotid produced by hemorrhage into the meningioma (253). In such artery territory; those with a mixed arterial supply but with cases, the patient may become comatose after the seizure. dominant feeders from the internal carotid artery; and those Additionally, frontal tumors may be associated with mental with a pure internal carotid artery supply, such as sellar me- changes, tumors in the parietal region may produce contralat- ningiomas or proximal tentorial meningiomas (258). Preop- eral motor or sensory abnormalities, and tumors in the occip- erative embolization facilitates removal of tumors in the first ital region may produce visual field defects (254). However, two groups. Most authors prefer to perform the embolization because these tumors are extrinsic, they usually produce several days before surgery. signs other than hemianopic visual field defects unless they Convexity meningiomas are in one of the most resectable become quite large. In fact, such tumors usually produce locations because of their distance from cranial nerves and symptoms and signs of increased (ICP) vital vessels (9,12). Nevertheless, ‘‘complete’’ tumor resec- long before they produce hemianopic field defects. Convex- tion is usually possible; recurrences still occur (66,177,178). ity meningiomas are unusual in children but have been re- It is likely that some of these recurrences develop from frag- ported (255). ments of tumor that are left behind at the time of surgery; Convexity meningiomas are easily diagnosed using stan- however, Borovich and Doron excised strips of dura sur- dard CT scanning or gadolinium-enhanced MR imaging, rounding 14 consecutive convexity meningiomas and found usually supplemented with angiography (225–227) (Figs. that all contained small aggregates of meningotheliomatous 30.17 and 30.18). cells (259,260). These cells could eventually produce a new Symptomatic convexity meningiomas should undergo meningioma that would be mistaken for a recurrence of the surgical removal. This may be facilitated by preoperative original meningioma. Thus, what appear to be recurrences

Figure 30.17. Neuroimaging of convexity meningioma in parietal region. A, Unenhanced computed tomography (CT) scan, axial view, shows loss of normal features of the parietal region on the right side, obliteration of the right lateral ventricle, and changes consistent with cerebral edema. B, After intravenous injection of iodinated contrast material, an axial CT scan at same level shows a large, dural-based, enhancing mass compressing the right parietal lobe. (Figure continues.) 1498 CLINICAL NEURO-OPHTHALMOLOGY

Figure 30.17. Continued. C, T2-weighted axial magnetic resonance (MR) image shows well-circumscribed mass that is hyperintense compared with surrounding brain. The mass compresses the right parietal lobe and obliterates the right lateral ventricle. Note marked hyperintensity of brain anterior and medial to the lesion, consistent with cerebral edema. D, T1-weighted axial MR after intravenous injection of paramagnetic contrast material shows homogeneous enhancement of the mass. Note obliteration of the right lateral ventricle.

may be separated into true local recurrences (regrowth of tumor within the limits of the previous resection) and false regional recurrences (which in fact represent new tumor growth outside the previous craniotomy site and are not re- currences at all). Borovich and Doron suggested that re- gional multicentricity may play a role in the ‘‘recurrences’’ of convexity meningiomas and meningiomas in other loca- tions that are thought to have been completely removed (259,260). They recommended that a wide resection of dura around meningiomas be performed at the time of surgery. The results of Ja¨a¨skela¨inen support this recommendation (76). Ja¨a¨skela¨inen found that meningiomas were more likely to recur after their dural insertion was coagulated than after it was excised. The results of Mirimanoff et al. suggest that, at least for convexity meningiomas, a 5-year period without evidence of recurrence has virtually no meaning and that one must wait at least 10 years after surgery before considering the patient ‘‘cured’’ (178). Because meningiomas are slow- growing, however, even a 10-year period may be too short. Postoperative radiation treatment should be considered in cases of incomplete resection or recurrence (9,10,178,261). Falx and Parasagittal Meningiomas

Figure 30.18. Neuroimaging appearance of convexity meningioma in the Meningiomas of the falx originate in the falx itself, occipital region. T1-weighted sagittal image after intravenous injection of whereas parasagittal meningiomas develop in the recess be- gadolinium shows a bilobed enhancing mass irregularly indenting the occip- tween the superior sagittal sinus and the falx. This latter site ital lobe. The mass extends inferiorly to the tentorium cerebelli. is one of the most common for a meningioma to occur (262). TUMORS OFTHE MENINGES AND RELATED TISSUES 1499

These tumors accounted for more than 20% of all meningio- the tumor, and whether a portion of the superior sagittal mas observed by the authors over a 30-year period. In 48 sinus is occluded by tumor. For purposes of discussion, such cases (17%), the tumor completely obliterated the superior tumors are usually separated into those involving the anterior sagittal sinus, whereas in 96 cases (34%), the sinus was par- third of the falx or sinus, those involving the middle third, tially obstructed. and those involving the posterior third. The symptoms and signs produced by parasagittal menin- Anterior meningiomas usually compress one or both fron- giomas and meningiomas arising from the falx depend on tal lobes and may become quite large before they are discov- the part of the brain compressed by the tumor, the size of ered (Fig. 30.19). By this time, symptoms and signs of long-

Figure 30.19. Neuroimaging appearance of anterior falx/parasagittal meningiomas. A, Axial computed tomography scan after intravenous injection of contrast shows a large enhancing parasagittal mass. B, In another case, a T1-weighted axial image after intravenous injection of gadolinium shows a large enhancing mass on both sides of the falx cerebri. Note changes in surrounding brain consistent with previous surgery and peritumoral edema. C, T1-weighted enhanced coronal image shows not only the mass but also enhancement of a thickened falx superior to the lesion. 1500 CLINICAL NEURO-OPHTHALMOLOGY standing increased ICP may be present (263). is torial junction are very rare and are characterized (275,276) the most prominent symptom (264). Other patients develop by headache, gait disturbance, and early . Cra- deterioration of mental function and changes in behavior nial nerve involvement (V, VI, VII, and VIII), homonymous (264–266). Seizures, as well as motor and sensory symp- hemianopias, upgaze limitation, hemiparesis, dysarthria, uri- toms, may also occur. At presentation, many patients have nary incontinence, and mental deterioration may also occur unilateral pyramidal signs and papilledema. In the study per- (276,277). Parasagittal meningiomas and meningiomas of formed by Giombini et al., almost 50% of the patients had the falx on occasion produce visual field defects that are papilledema when they were first examined on admission falsely localizing; central , peripheral constriction, to the hospital (264). Ataxia, tremor, and incontinence of noncongruous hemianopia, and bitemporal hemianopia have urine may also be present, and neuropsychological testing been described (271). often demonstrates evidence of disturbances in mentation The diagnosis of parasagittal meningiomas and meningio- (267). mas of the falx may be obtained using CT scanning, but Anterior falx meningiomas should be considered in the enhanced MR imaging is preferred (225–227,276). Conven- differential diagnosis of Parkinson’s disease, particularly tional or MR angiography is typically performed because when there is an associated alteration in consciousness meningiomas in this location have variable vascularity and (268,269). tend to obstruct the superior sagittal sinus. The middle third of the falx or sagittal sinus is the most The optimal treatment of a parasagittal or falx meningi- common location for parasagittal and falx meningiomas. Tu- oma is surgical excision, preceded in some cases by emboli- mors involving this region most frequently produce jackso- zation (256–258). When there is evidence of increased ICP, nian seizures starting in the contralateral leg (270). With consideration should be given to reconstructing the sinus at enlargement of the tumor, a sensory or motor neurologic the time of surgery if possible (262,274). If this is not possi- deficit may develop contralaterally or may even affect both ble, the patient should be treated postoperatively for the lower extremities. Of 197 patients with meningiomas in the pseudotumor cerebri syndrome by either medical or surgical region of the middle third of the falx or sagittal sinus who means. Visual field deterioration may occur despite tumor were evaluated by Giombini et al., 160 (81%) had unilateral removal if the increased ICP is not relieved. pyramidal signs (264). According to Dandy, tumors in this The operative mortality for patients with parasagittal and region are usually small when they produce neurologic falx meningiomas is less than 10% in most series (264). symptoms and signs (270). For this reason, they rarely cause After removal of these tumors, many patients have a marked headache or increased ICP. Only 25% of patients in the series improvement in their neurologic status (278). Not only is reported by Giombini et al. had (270). ICP normalized, but also patients with dementia usually re- Meningiomas that involve the posterior third of the falx cover, and disturbances in behavior resolve (265,266). In are the least common but the most likely to cause visual addition, less than 50% of patients with preoperative seizures symptoms. They may produce unilateral or even bilateral continue to experience seizures postoperatively (264,279). homonymous hemianopic or quadrantanopic field defects Giombini et al. reported that only 11 of 195 patients (5.6%) from pressure on one or both occipital lobes (271). They were completely disabled after removal of their tumors, also may produce seizures characterized by unformed vi- whereas 92 (47%) had no disability (264). sual hallucinations or transient homonymous hemianopia Meningiomas of the falx and parasagittal region are more (264,272). Some patients develop astereognosis and hemian- difficult to excise completely than convexity meningiomas. esthesia. In addition, when such tumors obstruct the posterior The recurrence/progression rate is therefore higher for these third of the superior sagittal sinus, they may produce the tumors than for meningiomas in all other locations except syndrome of pseudotumor cerebri (273). Increased ICP in the skull base. Postoperative radiation therapy is recom- such patients may occur from a direct mass effect or from mended to delay recurrence (66,176–178,264,280). destruction or occlusion of the posterior third of the superior sagittal sinus. In the latter group of patients, if the true cause Middle Cranial Fossa Meningiomas of the papilledema is not recognized and the tumor is simply removed, the ICP will remain elevated and the patient may Meningiomas in the middle cranial fossa are relatively eventually develop irreversible visual loss from postpapille- uncommon. The ocular signs include unilateral or bilateral dema optic atrophy (274) (Fig. 30.20). swelling sometimes associated with visual loss Meningiomas that arise in the falx may involve the tento- and optic atrophy, cranial neuropathies, and homonymous rium cerebelli, or vice versa. Tumors involving the falcoten- hemianopia or . The optic disc swelling is

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Figure 30.20. Pseudotumor cerebri syndrome caused by obstruction of the posterior superior sagittal sinus by a small meningi- oma. The patient was a 37-year-old woman who developed headaches and transient visual observations. When she was initially examined, visual acuity was 20/20 OU, but visual fields showed nasal constriction. A and B, Bilateral chronic papilledema is present. C, Computed tomography (CT) scan at the level of the lateral ventricles shows no evidence of ventricular dilation. D, CT scan at the level of the superior sagittal sinus shows a small posterior parasagittal meningioma. There is no significant mass effect from the tumor. E and F, Arteriovenous and venous phases of the cerebral angiogram show obstruction of a portion of the superior sagittal sinus by the tumor, which can be identified as an avascular region. TUMORS OFTHE MENINGES AND RELATED TISSUES 1501 1502 CLINICAL NEURO-OPHTHALMOLOGY usually related to increased ICP (i.e., papilledema), although mors cannot be totally removed. As a result, olfactory groove there may be indirect compression of the ipsilateral optic meningiomas have one of the highest rates of recurrence/ nerve as well. The oculomotor and trigeminal nerves are progression (66,177,178). Because these tumors have a sig- often affected (281–283). nificant tendency to recur or progress, postoperative radia- Gross total removal of meningiomas in the middle fossa tion therapy should be considered (241,243,280,298). is possible in most cases, promoting a low recurrence rate. These tumors usually invade the surrounding dura, so care Optic Nerve Sheath Meningiomas should be taken to remove or coagulate the adjacent dura to Optic nerve sheath meningiomas are usually separated reduce the risk of recurrence (66). into two types. Primary meningiomas arise from the cap cells of the arachnoid surrounding the intraorbital or, less Olfactory Groove Meningiomas commonly, the intracanalicular portions of the optic nerve. The vast majority (greater than 90%) are secondary meningi- These tumors account for about 10% of all intracranial omas that arise intracranially, usually from the sphenoid meningiomas and for about 3% of all intracranial tumors ridge, , or olfactory groove, and subse- (8,177,284,285). Olfactory groove meningiomas arise from quently invade the optic canal and orbit by extending be- arachnoid cells near the olfactory groove and the crista galli. tween the dura and arachnoid of the optic nerve in these Although they arise in the midline, they frequently grow regions. The term ‘‘optic nerve sheath meningioma’’ thus toward one side. These meningiomas almost always attain does not connote a definitive site of origin. Nevertheless, a considerable size before they are discovered because the once this type of tumor gains access to the subdural space area in which they grow is both neurologically and visually of the intracanalicular or intraorbital optic nerve, the tumor ‘‘silent.’’ The first clinical symptom is a reduction of the grows up or down the sheath, invading the dura and obliterat- sense of smell on one or both sides that eventually leads to ing the pial blood supply. In some instances, the tumor encir- (285). An intracranial mass is often not suspected cles the optic nerve without invading it. In other cases, the until the patient develops symptoms and signs of increased tumor invades the nerve by growing along the fibrovascular ICP, slowly progressive visual loss from pressure on the pial septa (299). Such a tumor may eventually surround and intracranial optic nerves or chiasm, or evidence of frontal obstruct the central retinal vein, central retinal artery, or both lobe compression (seizures, incontinence, or disturbances vessels. Rarely, a tumor breaks through the dural sheath of in memory, affect, and behavior) (285–290). These defects the optic nerve to invade other orbital structures. Less com- occur initially on the side of the tumor. In some cases, olfac- monly, a tumor that originates within the optic nerve sheath tory groove meningiomas grow between the optic nerves, in the orbit or optic canal may grow intracranially to involve forcing them against the internal carotid and anterior cerebral the optic chiasm, contralateral optic nerve, and internal ca- arteries. Compression of the temporal portions of the optic rotid artery. It may even invade the cavernous sinus or the nerves by these vessels produces bilateral nasal field defects sella turcica. (291). Less commonly, may occur when The symptoms and signs of optic nerve sheath meningio- hemorrhage develops (289). Optic atrophy occurs in mas depend in large part on whether they have arisen within 30–70% of patients with olfactory groove meningiomas the orbit, within the optic canal, or intracranially. (285,287). The atrophy may be unilateral or bilateral and is often asymmetric. Optic Nerve Sheath Meningiomas, Primary The Foster Kennedy syndrome was described in 1911 and consists of the triad of optic atrophy, contralateral papille- These tumors arise from cap cells of the arachnoid sur- dema, and anosmia (or hyposmia) (Fig. 30.21). This syn- rounding the optic nerve in the orbit or optic canal. Their drome rarely occurs in a pure form (285,287,290,292–296). histology is that of syncytial or transitional meningiomas. One common variant of the Foster Kennedy syndrome is As with other meningiomas, primary optic nerve sheath me- characterized by bilateral visual loss with unilateral optic ningiomas often develop in middle-aged women, but they disc swelling. In other cases, there is bilateral visual loss occasionally occur in children as well (300–308). Dutton et associated with bilateral disc edema from both bilateral optic al. reported a mean age of 41 years and less than 4% younger nerve compression and increased ICP. The optic atrophy than 20 years old (309,310). Primary optic nerve sheath me- does not consistently occur on the side of the lesion (290). ningiomas may also occur in patients in their sixth decade The Foster Kennedy syndrome is a triad that includes anos- and beyond (304–307). mia. Acute ischemic that is sequential can Most primary optic nerve sheath meningiomas are unilat- produce unilateral optic disc swelling with optic atrophy on eral, but bilateral cases do occur, typically before the age of the contralateral side, but the presence of anosmia in this 20 (311,312). Dutton reported a bilateral rate of 6%; of those, setting is virtually pathognomonic of a lesion involving the 60% were canalicular (310). Some patients with apparent olfactory groove area, sphenoid ridge, or, in rare cases, the bilateral optic nerve sheath meningiomas are found at crani- frontal lobes (293,297). otomy to have involvement of the planum sphenoidale be- Once an olfactory groove meningioma is suspected, its tween the two optic canals (313). In such cases, there re- presence is easily confirmed with MR imaging augmented mains some question of whether the lesions were truly with MR angiography or traditional angiography. Treatment multifocal or if there was not simply spread of tumor from of olfactory groove meningiomas is surgical, but many tu- one site into both canals. Gadolinium-enhanced MR imaging TUMORS OFTHE MENINGES AND RELATED TISSUES 1503

Figure 30.21. Foster Kennedy syndrome in a 56-year-old woman with an olfactory groove meningioma. The patient had 20/200 vision in her right eye with a constricted visual field and a relative afferent pupillary defect. Visual acuity in the left eye was 20/15 with normal color perception and a normal visual field except for an enlarged blind spot. A, The right optic disc is atrophic, and the retinal arteries are narrow. B, The left optic disc shows changes consistent with chronic swelling. C, Coronal computed tomography scan after intravenous injection of iodinated contrast shows a huge mass arising from the and filling the anterior fossa on both sides. allows a more accurate preoperative clarification as to gers vision or worse (310). In most of these patients, there is whether tumor crossover is present. evidence of an underlying optic neuropathy (e.g., diminished Patients with optic nerve sheath meningiomas usually color vision or at least relative desaturation of red compared complain initially of decreased or blurred vision in their af- with the fellow eye; relative afferent pupillary defect) (307). fected eye, although at the time of examination, the vision Other patients complain only of transient visual loss lasting may be 20/20 or better (304,307). According to Dutton, 97% a few seconds, similar to that described by some patients of patients have visual loss at presentation, but 45% have with increased ICP and papilledema (307,310,314,315). vision of 20/40 or better and less than 25% have count fin- Transient visual obscuration may occur with eye movement 1504 CLINICAL NEURO-OPHTHALMOLOGY

the optic disc of the affected eye may be swollen, pale, or normal in appearance. The optic disc is usually swollen when the tumor surrounds or compresses the intraorbital portion of the nerve (303,304,307) (Fig. 30.22). When the patient has visual complaints and evidence of an underlying optic neuropathy, the diagnosis is rarely in question; however, in some cases, the examination is relatively unremarkable. The most common cause of unilateral optic disc swelling associ- ated with ‘‘normal’’ visual acuity is an orbital process, not increased ICP. Although many patients with optic nerve sheath meningi- omas involving the orbital portion of the optic nerve initially have relatively good visual function, they gradually lose vi- sion with time. As this occurs, the optic disc swelling begins to resolve, and optociliary shunt vessels may appear on the surface of the disc (303,307,312,315–322) (Figs. 30.23 and 30.24). These vessels shunt venous blood from the retinal to the choroidal circulation, thereby allowing egress via the Figure 30.22. Swelling of the left optic disc in a 35-year-old woman with superior or inferior ophthalmic veins rather than via the cen- a primary optic nerve sheath meningioma. The woman had noted for several tral retinal vein. When optociliary shunt vessels are acquired, months that the vision in her left eye was ‘‘not normal.’’ Although visual acuity in the left eye was 20/20, there was slight red desaturation in that they are pathognomonic of chronic central retinal vein eye compared with the right eye, a slightly constricted left visual field, and compression. The triad of visual loss, optic atrophy, and a left relative afferent pupillary defect. The patient was found to have a optociliary shunt veins is characteristic of an optic nerve primary optic nerve sheath meningioma that extended from the to sheath meningioma; however, optociliary shunt vessels asso- the intracranial portion of the optic canal. ciated with visual loss and optic atrophy also occur in associ- ation with optic nerve gliomas and meningiomas of the sphe- noid wing (317). The shunt vessels are seen in only a third (gaze evoked amaurosis) (307). Proptosis (which is usually of optic nerve meningiomas. This may be in part because 2–5 mm) and (most commonly limitation of up- they often become subtler or resolve as atrophy progresses gaze) tend to develop after the optic nerve dysfunction (310). (303,305,306,310). Not all patients with primary optic nerve sheath meningio- In patients with primary optic nerve sheath meningiomas, mas have swollen optic discs. When the tumor originates at

Figure 30.23. Appearance of optociliary shunt vessels. A, Optociliary shunt vessels in a 55-year-old woman with a spheno- orbital meningioma. B, Drawing of optociliary shunt vessels showing that they represent veins that shunt blood between the retinal and choroidal venous circulations. Retinal venous blood cannot exit the eye via the central retinal vein because it has been compressed (e.g., by tumor). Collateral channels that normally exist between the retinal and choroidal venous circulations subsequently enlarge so that the retinal venous blood is shunted to the choroidal venous circulation to exit the eye via the vortex veins. TUMORS OFTHE MENINGES AND RELATED TISSUES 1505

Figure 30.24. Progressive appearance of optociliary shunt vein in a patient with a primary optic nerve sheath meningioma of the right eye. A, Optic disc swelling is present. The optociliary vessel is barely visible (arrow). The patient’s visual acuity in this eye was 20/20. B, One year later, visual acuity in the right eye has dropped to 20/100. The disc swelling has resolved, and optic atrophy is now present. The optociliary shunt vein is prominent (arrow).

the apex of the orbit or within the optic canal, there is slowly meningioma (335–337). Four percent of optic nerve sheath progressive visual loss without orbital signs, usually with a meningiomas demonstrate focal tumor invasion of the optic normal-appearing optic disc (323). Although such patients disc, , choroid, and (310). almost always have evidence of an underlying optic neuropa- The treatment of primary optic nerve sheath meningiomas thy, the physician may overlook this. With time, the disc is controversial because they rarely represent a life-threaten- becomes pale, but until it does, the true cause of the visual ing process (305,306,338). Surgical excision usually results loss may be obscure (324). Before the availability of high- in complete visual loss (339,340). This occurs because the resolution neuroimaging, these tumors were almost impossi- tumor completely surrounds (and occasionally invades) the ble to detect without craniotomy, although occasionally optic nerve and is so diffuse that it cannot be peeled away characteristic changes in the bone of the optic canal could (Fig. 30.26). In addition, during the attempt to remove such be identified by complex-motion tomography (325–329). lesions, there may be surgical damage to the pial vasculature, Once a primary optic nerve sheath meningioma is sus- resulting in loss of remaining vision (341). Patients with pected, the diagnosis can usually be established using gado- diffuse optic nerve sheath meningiomas and progressive vis- linium-enhanced MR imaging now rather than CT imaging ual loss should receive radiation to preserve visual function with contrast (9,12,305,306,330–333) (Fig. 30.25). Other (342–345). If severe or intracranial exten- features can be seen on either imaging modality and include tubular enlargement of the nerve, bulbous enlargement of sion is present, then complete removal of the tumor and optic the optic nerve at the apex with distal tubular enlargement, nerve is indicated via craniotomy. Meningiomas in children and the appearance of the optic nerve on coronal section as and young adults are more aggressive and life-threatening a hypodense area surrounded by a more dense peripheral and warrant earlier and more complete intervention. ring. Marked, homogenous enhancement with gadolinium is In some patients with tumors confined to the optic canal, also typical, and some tumors have a marked cystic compo- microsurgical decompression of the canal may stabilize or nent (334). On very rare occasions, small tumors located improve visual function for many years, but such tumors within the optic canal remain impossible to detect using neu- often continue to grow (346–348). This procedure remains roimaging procedures. Such lesions can be identified only an alternative in the patient who undergoes an exploratory during exploratory craniotomy, but they should be suspected craniotomy for unexplained visual loss and is found to have in any patient with slowly progressive, unilateral loss of an optic nerve sheath meningioma surrounding the optic vision associated with signs of optic neuropathy. In addition, nerve within the optic canal (349). the presence of enlarged, aerated posterior ethmoid and When there is evidence of intracranial spread of tumor sphenoid sinuses, a condition known as pneumosinus dila- across the planum sphenoidale in a patient with a primary tans, is thought by some authors to be pathognomonic of a optic nerve sheath meningioma and useful visual function, 1506 CLINICAL NEURO-OPHTHALMOLOGY

Figure 30.25. Neuroimaging appearance of optic nerve sheath meningiomas. A, Computed tomography (CT) scan, axial view, shows diffusely enlarged right optic nerve. Note calcification along the nerve. B, CT scan, coronal view, in the same case shows that the right optic nerve is enlarged and calcified. C, In a different patient with an optic nerve sheath meningioma, an enhanced axial CT scan shows that the left optic nerve is diffusely enlarged and surrounded by calcification. D, T2-weighted magnetic resonance image, sagittal view, in a third patient shows the outline of the left optic nerve surrounded by the tumor within the orbitalapex. a craniotomy should be performed to remove the intracranial In adults, observation was previously recommended until portion of tumor, thereby preventing tumor spread to the significant visual loss developed (299,304,310,341). In such contralateral optic nerve. In such cases, the tumor surround- patients, the progression of visual loss may be extremely ing the intracanalicular and orbital portions of the affected slow, occurring over many years. However, the efficacy of optic nerve is usually left alone, although the optic canal radiation has been demonstrated, and such patients should may be opened. undergo regular clinical examinations every 3–6 months and TUMORS OFTHE MENINGES AND RELATED TISSUES 1507

Figure 30.26. Optic nerve sheath meningioma. A, Macroscopic appear- ance showing intraorbital portion of optic nerve (small arrowheads) com- pressed by meningioma (M) that remains confined within the dural sheath of the nerve (large arrowheads). B and C, Cross-sections through the optic nerve of a patient with an optic nerve sheath meningioma show the tumor surrounding and compressing the nerve but confined between the pia arach- noid and the dural sheath. Note numerous psammoma bodies. repeat CT scanning or MR imaging every 6–12 months. almost always required to try to prevent the tumor from If visual field progression is demonstrated, external beam damaging other intracranial structures, including the contra- radiation (4,500–5,000 rads) or fractionated stereotactic ra- lateral optic nerve, optic chiasm, internal carotid artery, pi- diation therapy should be considered (189,342,350,351). tuitary gland, and cavernous sinus. A frontotemporal craniot- omy should be performed and complete tumor removal Optic Nerve Sheath Meningiomas, Secondary without injury to vital structures attempted. Such a procedure will necessitate removal of the involved optic nerve. If resid- These tumors originate intracranially, usually in the re- ual tumor remains, postoperative radiation therapy should gion of the planum sphenoidale or tuberculum sellae, and be performed to minimize the risk of progression and recur- gradually extend into the optic canal. In many cases, it may rence. be difficult, even at surgery, to determine whether a meningi- oma began in the posterior orbit or optic canal and then Orbital Meningiomas spread intracranially, or whether the tumor began intracrani- ally and spread into the optic canal. In both cases, there is Optic nerve sheath meningiomas may originate in or ex- slowly progressive visual loss associated with evidence of tend into the orbit, but these are different from the orbital an optic neuropathy and a normal or pale optic disc. MR meningiomas that originate from ectopic meningeal tissue imaging with gadolinium should be performed to identify that is pinched off within the orbit during intrauterine devel- and follow these lesions. opment (305,306). Such tumors may occur outside the mus- The problems inherent in treating secondary optic nerve cle cone or even adjacent to the optic nerve, but in an extra- sheath meningiomas are similar to those that relate to pri- dural location (339,352,353). mary optic nerve sheath meningiomas. Because there is an It is important to recognize the difference between orbital intracranial component, some amount of tumor removal is meningiomas of this type and those that are located within 1508 CLINICAL NEURO-OPHTHALMOLOGY the dural sheath of the optic nerve. The latter type of tumor by Hart and Lillehei, presenting symptoms included hearing is almost never amenable to complete removal with preser- loss, unsteadiness of gait, pulsatile tinnitus, episodic vertigo, vation or restoration of vision, whereas the type of orbital and trigeminal hypesthesia or trigeminal neuralgia (19,369). meningioma encountered by Watson and Greenwood and Papilledema may be present in patients with increased Mark et al. may lend itself to microsurgical removal with ICP and may evolve into optic atrophy if the lesion is not subsequent improvement in vision (339,352). Fine-needle removed. In other patients, postpapilledema optic atrophy is aspiration biopsy can be helpful in establishing the diagnosis already present when the tumor is first discovered. (354). Because CPA meningiomas are intradural, the symptoms and signs are similar to those that occur in patients with Posterior Fossa Meningiomas acoustic schwannomas. The clinical differentiation between Meningiomas that arise within the posterior fossa may these two lesions—which account for 75–90% of all tumors originate in several different locations, including the tento- in this region (acoustic schwannomas, 70–80%; meningio- rium cerebelli, petrous ridge, clivus, foramen magnum, and mas, 6–8%)—may be difficult. Acoustic schwannomas are fourth ventricle (19). Meningiomas in this region are rarely more common in men than in women. The hearing impair- cystic or globular but instead typically grow in an en-plaque ment associated with acoustic schwannomas is more severe, pattern, with the site of attachment often extending from but meningiomas cause facial pain or numbness more often one area to another. Large (CPA) than do acoustic schwannomas (358,361–364,367). Never- meningiomas, for instance, commonly extend into the clival theless, the diagnosis usually rests with neuroimaging, and tentorial regions. Tumors classified by some authors as particularly CT scanning, MR imaging, and cerebral angiog- CPA meningiomas may be considered clival tumors by oth- raphy (19,225–227,364,367,370–373). Evidence of calcifi- ers. In addition, there are occasionally meningiomas that cation or hyperostosis of the petrous apex strongly favors arise in the posterior fossa but do not appear to have any meningioma, whereas bony changes of the internal auditory dural attachment (355,356). canal rarely occur with meningiomas but frequently occur These tumors may also arise from the fourth ventricle or with acoustic schwannomas. MR angiography is excellent within the . These considerations are impor- for assessing dural venous sinus flow and arterial encase- tant when comparing presenting symptoms and signs as well ment by tumor (19). Despite advanced neuroimaging, the as morbidity and mortality after treatment (19,357). In the correct diagnosis may not be made until craniotomy is per- sections that follow, we have separated posterior fossa me- formed (362). Other studies that may be performed include ningiomas by location within that region to emphasize their brain stem auditory evoked responses, blink-reflex testing, different presentations and the results of treatment, but we and electronystagmography. Although these studies are usu- are aware that such a classification is somewhat arbitrary. ally abnormal in patients with CPA meningiomas, they usu- ally do not help to distinguish these tumors from acoustic Cerebellopontine Angle Meningiomas schwannomas (374). Although less than 20% of all meningiomas occur in the Tumor location, preoperative cranial nerve status, tumor posterior fossa, half of these are located in the CPA vascularity and blood supply, and the presence of dural ve- (19,358,359). Meningiomas that arise in the CPA occur with nous sinus invasion influence surgical planning. The surgical a frequency second only to that of acoustic schwannomas approaches to CPA and clival meningiomas have radically (360,361). In contrast to acoustic neuromas, CPA meningio- changed because of the tremendous emphasis on minimally mas are almost always unilateral (361–363). invasive skull base surgical techniques. The approaches in- Most patients with CPA meningiomas present with more clude the subtemporal, petrosal, retrolabyrinthine, translaby- than one symptom. Hearing loss, the most common symp- rinthine, transmastoid, and posterior fossa—and all combi- tom, is usually slowly progressive and accompanied by ver- nations of the above. The size, location, and cranial nerve tigo, imbalance, or tinnitus (364–367). Other symptoms in- involvement and the surgeon’s experience all determine clude an alteration in facial sensation, facial pain, and which approach is optimal. Overall morbidity has been re- headache. Rare patients complain of diplopia or . duced, and complete resections are now possible (19,361, Symptoms of increased ICP occur if the tumor obstructs the 362,375–379). normal flow of CSFor occludes one of the dural sinuses. If complete resection is not possible with acceptable mor- In the latter setting, the presentation simulates that of pseu- bidity, stereotactic radiosurgery and conventional external dotumor cerebri and is identical to that which results when beam radiation therapy are reasonable treatment options. a falx meningioma occludes the superior sagittal sinus (368). Sekhar and Jannetta favored radiation therapy primarily for Neurologic signs that occur in patients with CPA meningi- angioblastic and invasive meningiomas (362). omas include neurosensory hearing loss, vestibular nystag- The prognosis for patients with meningiomas of the CPA mus, trigeminal sensory neuropathy (especially decrease in is reasonably good. Complications such as hemorrhage, in- or loss of the corneal reflex, but also typical or atypical fection, cranial neuropathies, and rarely gaze-evoked tinni- trigeminal neuralgia), and cerebellar ataxia. Other patients tus have been described after removal of a CPA meningioma have ocular motor nerve dysfunction, facial weakness, lower (362,375,380). With these tumors, as with meningiomas cranial neuropathies, appendicular weakness, altered mental elsewhere, ‘‘complete’’ surgical removal does not guarantee status, or a combination of these deficits. In a series reported that recurrence will be prevented (259,260). TUMORS OFTHE MENINGES AND RELATED TISSUES 1509

Meningiomas of the Clivus Stage I tumors are separate from the pia mater with a distinct subarachnoid plane. In stage II, invasion or compression is These rare, slowly growing tumors arise from the area of present and the subarachnoid plane is lost. The pia is invaded the synchondrosis of the sphenoid and occipital bones on and edema, demyelination, or gliosis may be present in stage the clivus blumenbachii (381,382) (Fig. 30.27). The initial III. Sekhar et al. found that tumor size, presence of edema, symptom is typically headache, followed by disturbance in brain stem compression, vascular encasement, and identifi- gait, difficulty with hearing, vertigo, or visual difficulties, cation of an arachnoidal cleavage plan were all helpful in particularly double vision. Papilledema, loss of hearing, and surgical planning and preoperative counseling (377). ataxia are common findings. Cranial neuropathies are also The treatment for meningiomas of the clivus is surgical quite common, with the trigeminal, abducens, facial, and removal. Until the 1990s, the surgical mortality was as high vestibulocochlear nerves being affected most frequently. as 50%. Since 1990, the mortality rate has dropped to less Hemiparesis or monoparesis (contralateral or ipsilateral to than 1% in most series (378,383). Tumor size and location the lesion) and less commonly bilateral somatic motor defi- determine the surgical approach used, particularly whether cits may develop (383). it lies in the midline or to one side (382). Although complete CT scanning, with bone windows, will demonstrate the removal is rarely possible, meningiomas of the clivus are so mass and associated bony changes. Meningiomas do not slow-growing that if the bulk of the tumor can be removed at typically produce as much bone destruction as chordomas. a relatively early stage without producing major neurologic However, MR imaging with gadolinium is the best way to deficits, most patients do quite well for many years (382). differentiate meningiomas from chordomas; meningiomas In a series by Sekhar et al., factors such as tumor characteris- brightly enhance in a homogeneous fashion. Angiography tics and operative difficulties were analyzed in 75 patients may be of some value in assessing blood supply pending with clival meningiomas (377). Large tumor size was predic- surgical removal, but from a differential viewpoint, MR im- tive of temporary neurologic deterioration, and blood supply aging and CT scanning are the most valuable studies by the basilar artery was most predictive of permanent neuro- (377,382). Sekhar et al. suggested that these tumors be logic deficits. In this series, 40% of patients experienced staged based on their appearance on MR imaging (377). mild neurologic deterioration (memory loss, mild hemipare-

Figure 30.27. Meningioma of the clivus. A, Diagram of a tumor that produced a number of neurologic symptoms and signs, including pathologic laughing and crying. B, T2-weighted magnetic resonance image, axial view, in another patient shows marked compression of the brain stem from a left-sided clivus meningioma. (A, From Cantu RC. Importance of pathological laughing and/or crying as a sign of occurrence or recurrence of tumor lying beneath the brainstem. J Nerv Ment Dis 1966; 143Ϻ508–512.) 1510 CLINICAL NEURO-OPHTHALMOLOGY sis, and facial weakness), whereas only 4% had severe neuro- tion experience a marked recovery after surgery, even if logic decline (hemiplegia, quadriparesis, or depressed senso- brain stem decompression alone is accomplished. rium). Overall, only 27% of patients had a persistent neurologic dysfunction. Meningiomas of the Fourth Ventricle

Meningiomas in the Foramen Magnum Meningiomas in the fourth ventricle are extremely rare (393). These tumors often have no dural attachment and Meningiomas are the most common benign tumors in the appear to arise in the region of the choroid plexus (355). foramen magnum region (384,385,386). Foramen magnum There appears to be no characteristic clinical picture that meningiomas are rare in children but occur in all other age allows differentiation from the more common ependymomas groups (384,386). or choroid plexus papillomas that occur in this region. If the The most frequent presenting complaint of patients with meningioma is small, it may produce no symptoms (394), meningiomas in the foramen magnum is suboccipital (cervi- but most patients develop symptoms and signs of increased cal) neck pain exacerbated by coughing and straining ICP when the tumor blocks the flow of CSFin the fourth (384–386). In some cases, the pain radiates into one arm. ventricle. In other cases, the tumor extends into the cerebel- Other symptoms include dysesthesias, particularly of the arm lum to produce nonspecific cerebellar symptoms and signs. and hand. These symptoms may be unilateral or bilateral. The treatment of meningiomas located with the fourth ventri- Occasionally, the leg or face is affected. Some patients com- cle is surgical, and complete removal is often possible with- plain of difficulties with walking. out significant morbidity or mortality. The neurologic examination may be initially normal, but progressive neurologic signs such as extremity weakness and atrophy of the intrinsic hand muscles may develop. These Meningiomas of the Tentorium Cerebelli patients may develop hyperreflexia with extensor plantar re- These tumors are so classified because their predominant sponses. Cerebellar dysfunction is quite rare; the only cranial site of attachment is to the tentorium cerebelli. Although nerve affected is the spinal accessory nerve. is they are somewhat rare, representing only 2.7% of meningio- often present; it may be horizontal, torsional, downbeat, up- mas seen at the Mayo Clinic, several large series have been beat, or rarely periodic alternating nystagmus. Horner’s syn- reported (8,395–401). drome occurs in some patients (384). Some tentorial meningiomas are located completely Meningiomas in the foramen magnum grow slowly and above or below the tentorium, whereas others extend both may produce symptoms for many years before neurologic above and below that structure. Of 132 cases described in signs appear or are detected (386,387). The differential diag- nosis includes cervical spondylosis, multiple sclerosis, syrin- the literature, 47 were classified as supratentorial, 48 were gomyelia, intramedullary tumor, and Chiari malformation. infratentorial, and 37 had both supra- and infratentorial com- Patients who present with atrophy of the intrinsic hand mus- ponents (8,397,398). Supratentorial meningiomas tended to cles initially may be thought to have a carpal tunnel syn- produce symptoms and signs that developed rapidly and drome. suggested a lateralized cerebral mass lesion. In contrast, Gadolinium-enhanced MR imaging is the diagnostic study infratentorial meningiomas produced several different of choice (10,388,389). MR angiography and standard angi- syndromes: obstructive hydrocephalus, trigeminal nerve ography provide information regarding the degree of vascu- dysfunction and vestibulocochlear neuropathy, or gait dis- larity of the meningioma (10,390). turbance (398,402,403). Although removal of meningiomas, particularly those that Most tentorial meningiomas are readily identified using are ventral in the foramen magnum, may be quite difficult, CT scanning, but enhanced MR imaging is more sensitive advances in skull base techniques greatly facilitate complete (10,225–227). MR imaging not only permits identification excision and allow preservation of neurologic function. By of the lesion but also allows precise localization through the using an extreme lateral inferior transcondylar exposure, tu- use of axial, coronal, and sagittal images. mors ventral to the brain stem involving the anterior rim of The treatment of tentorial and pineal region meningiomas the foramen magnum may be completely removed with little is primarily surgical. In the past, removal of these tumors or no morbidity (391,392). If residual tumor remains, en- was associated with a significant mortality, ranging from hanced MR imaging is an excellent modality for following 7% to 44% (8,397,398). Surgical excision is now safe (404). these patients. If recurrence or progression occurs, reopera- The postoperative complications that occur in patients with tion or stereotactic or external beam radiation may be consid- tentorial meningiomas are related to the size of the tumor ered. As might be expected, the best prognosis is in patients and its proximity to various neural and vascular structures whose tumors are diagnosed early, because functional recov- in the posterior fossa. ery is largely related to preoperative neurologic status. Thus, patients who are neurologically intact before surgery tend to Sphenoid Wing Meningiomas remain that way after surgery, whereas patients with severe neurologic deficits are often left with serious handicaps. The sphenoid ridge curves outward from the anterior cli- Nevertheless, some patients with severe neurologic dysfunc- noid process to join with the frontal and parietal bones of TUMORS OFTHE MENINGES AND RELATED TISSUES 1511

Figure 30.28. Three divisions of the sphenoid ridge from which meningi- omas originate can often be separated on the basis of clinical symptoms and signs. (From Cushing H, Eisenhardt L. Meningiomas. Springfield, IL: Charles C Thomas, 1938.) the skull at the pterion (Fig. 30.28). Although the sphenoid ridge is only about 5 cm long, tumors arising from it are usually separated into three groups because of the different symptoms and signs that they produce: tumors of the outer Figure 30.29. Magnetic resonance appearance of pterional meningioma. (pterional) ridge, tumors of the middle (alar) ridge, and tu- T1-weighted axial scan after intravenous injection of gadolinium shows mors of the inner (clinoidal) ridge (405,406). irregular thickening of the pterion (arrowhead). Note lack of significant enhancement. Meningiomas Arising from the Outer Sphenoid Wing and Pterion These tumors arise from the outer third of the sphenoid and signs identical with those produced by inner ridge tu- ridge, which is part of the greater wing of the sphenoid. mors. The symptoms are the same in nearly all cases. The Cushing and Eisenhardt described two types of tumors that patient develops slowly progressive, unilateral, nonaxial arise in this region, each with distinct symptoms: pterional (slightly downward) proptosis with periorbital edema (Fig. meningiomas and en-plaque meningiomas (8). 30.30). With time, usually over a period of several years, Pterional meningiomas grow from the pterion toward the a characteristic swelling in the ipsilateral temporal region sylvian fissure. These tumors occur with equal frequency in men and women. Unlike their en-plaque counterparts (dis- cussion following), they often become quite large before producing any symptoms or signs. Most patients eventually present with headache and have papilledema and other evi- dence of increased ICP. Some patients develop visual hallu- cinations, whereas others experience seizures, uncinate at- tacks, central facial paralysis, somnolence, or hyperreflexia. Dementia is not uncommon (265). All of these symptoms relate to pressure from the growing tumor on portions of the temporal and frontal lobes. By the time most pterional meningiomas are suspected, they are easily identified by almost all neuroimaging studies, although they are best defined by CT scanning or MR imag- ing (10,225–227) (Fig. 30.29). Early surgical resection is warranted. In most cases, symptoms and signs resolve or improve once the tumor is removed (265). En-plaque meningiomas in this region exert almost no pressure on the cerebral hemispheres. They form only a thin intradural layer of tumor. The bulk of the tumor infiltrates the dura and invades the bone of the sphenoid ridge, producing Figure 30.30. Appearance of a patient with an en-plaque meningioma marked thickening or hyperostosis (407). Despite their lat- of the left sphenoid ridge. Note axial proptosis and somewhat downward eral origin in the region of the pterion, they eventually spread displacement of the left eye. The patient also has characteristic swelling of medially across the sphenoid ridge to produce symptoms the left temporal fossa. 1512 CLINICAL NEURO-OPHTHALMOLOGY

Figure 30.31. En-plaque meningioma in the pterional region of the left sphenoid wing. The patient has 5 mm of left proptosis. Visual acuity was 20/20 OU, and there was no abnormality of eye movements, although both optic discs were mildly swollen. Note obvious swelling in the left temporal region. (From Huber A. Eye Signs and Symptoms in Brain Tumors. Ed 3. St Louis: CV Mosby, 1976.) develops from hyperostosis and thickening of the greater years. Eventually, they may enlarge sufficiently to invade wing of the sphenoid (8) (Fig. 30.31). As the tumor continues the orbit through infiltration of bone or extension into the to grow and the posterior orbital bones become increasingly superior orbital fissure and optic canal (Fig. 30.32), thus thickened, proptosis becomes severe and diplopia in lateral producing clinical features identical to those produced by gaze may develop. Loss of vision from optic nerve compres- meningiomas that arise from the inner third of the sphenoid sion is rare and occurs late. It is ultimately associated with ridge. Meningiomas that arise from the middle third of the optic disc pallor. Although optic disc swelling occasionally sphenoid ridge may also enlarge sufficiently to compress the occurs, it is directly related to compression of the orbital ipsilateral temporal lobe, producing seizures, homonymous portion of the optic nerve and is not papilledema caused by hemianopia, and eventually increased ICP. increased ICP. Rarely, thickening of the affected portion of the sphenoid ridge or enlargement of the intracranial soft Meningiomas Arising from the Inner Sphenoid Wing tissue component of the tumor compresses the frontal or temporal lobes, producing mental disturbances or seizures, Meningiomas that arise from the inner ridge of the sphe- but this is most unusual. noid are often described as arising from the lesser wing of The neuroimaging appearance of an en-plaque meningi- the sphenoid. Because of the proximity to the optic canal, oma is as characteristic. On CT scanning, erosion of the intracranial portion of the optic nerve, superior orbital fis- bone in the region of the pterion, with thickening of the sure, and cavernous sinus, these tumors often produce visual lateral orbital wall and roof as far posteriorly as the anterior symptoms and signs while still quite small (411–413). clinoid process, is typically present. The soft tissue compo- One characteristic syndrome of all inner sphenoid ridge nent of the tumor that may occur on the orbital and cranial meningiomas is that of unilateral, progressive loss of vision sides of the bone is best visualized with gadolinium- associated with signs of optic neuropathy (e.g., reduction of enhanced MR imaging. color vision, visual field defect, relative afferent pupillary En-plaque meningiomas in this region tend to be slowly defect, and atrophy of the optic nerve and peripapillary reti- growing tumors. Patients with contraindications to surgery nal nerve fiber layer). Because the tumor that produces the should be followed. Early surgical intervention is indicated loss of vision is usually small when visual symptoms begin, when symptoms and signs are minimal prior to optic nerve patients usually have no headache or other symptoms of damage. The intracranial soft tissue component can be re- an intracranial mass. They may not seek medical attention moved, and the bony component can be substantially re- because they have no symptoms except for mild visual field duced using a high-speed drill. The posterior lateral orbital loss in one eye. Unilateral visual loss may progress until the wall and orbital roof can be almost completely removed, patient has no useful vision in the eye and there is profound allowing decompression of the superior orbital fissure and optic atrophy. Bilateral reduction of visual acuity associated optic nerve and reducing or eliminating proptosis, at least with a variety of visual field defects in each eye (e.g., binasal temporarily (408). External beam radiotherapy is beneficial defects, central scotomas) may occur from damage to the in preventing or delaying recurrence of incompletely excised contralateral optic nerve. A temporal field defect may de- or recurrent tumors (242,243,247,280,409,410). velop in the otherwise asymptomatic contralateral eye from damage to the fibers of Wilbrand’s knee in the distal portion Meningiomas Arising from the Middle Third of the of the ipsilateral optic nerve, from involvement of the optic Sphenoid Wing chiasm, or from involvement of the ipsilateral optic tract. Because they arise from the middle third of the sphenoid If a tumor that causes unilateral progressive visual loss ridge, they usually produce no symptoms or signs for many becomes large enough, it will eventually produce increased TUMORS OFTHE MENINGES AND RELATED TISSUES 1513

Figure 30.32. Meningiomas arising from the middle third of the sphenoid ridge. A, Appearance of a 45-year-old man with a meningioma that arose from the middle third of the left sphenoid ridge and extended medially to infiltrate the left orbit through the left superior orbital fissure and optic canal. Note marked axial proptosis of the left eye. B, Macroscopic appearance of a meningioma that originated from the middle third of the sphenoid ridge in a 60-year-old man who died of the effects of hypertension. The view is of the inferior surface of the optic nerves, chiasm, and tracts. The tumor surrounds the left optic nerve and extends posteriorly to lie adjacent to the left side of the optic chiasm and the proximal portion of the left optic tract.

ICP. By this time, the ipsilateral optic nerve may be pro- found that it or one of its variants occurred most frequently foundly atrophic, and therefore papilledema may occur only in patients with meningiomas arising from the inner sphe- in the contralateral eye. When anosmia results from exten- noid ridge (8). sion of the tumor to the olfactory nerves, the syndrome is A second major group of visual symptoms and signs pro- called the Foster Kennedy syndrome (414). This syndrome duced by inner sphenoid ridge meningiomas relates to in- consists of a triad of unilateral optic atrophy, contralateral volvement of the cavernous sinus, superior orbital fissure, papilledema, and anosmia (Fig. 30.21). Even when anosmia and orbit. Such patients complain primarily of diplopia and is absent, unilateral optic atrophy and contralateral papille- proptosis (Fig. 30.33). In such cases, the abducens nerve is dema may be considered a variant of this syndrome. Al- most frequently affected, but the oculomotor and trochlear though the Foster Kennedy syndrome is most often equated nerves also may be damaged, and total ophthalmoplegia oc- with olfactory groove meningiomas, Cushing and Eisenhardt curs in rare cases. When the tumor also compresses the oph-

Figure 30.33. Right proptosis with eyelid swelling and down- ward displacement of the right globe in a woman with a meningi- oma of the inner third of the right sphenoid ridge. Visual acuity in the right eye was only light perception, and the motility of the eye was limited in all directions. There was optic atrophy in the right eye. The left eye was normal. (From Huber A. Eye Signs and Symptoms in Brain Tumors. Ed 3. St Louis: CV Mosby, 1976.) 1514 CLINICAL NEURO-OPHTHALMOLOGY thalmic division of the trigeminal nerve, a patient with diplo- may be an associated homonymous hemianopia or quad- pia also complains of pain, numbness, or dysesthesia in the rantanopia (411). cutaneous distribution of the branches of that nerve. In such MR imaging is diagnostic, but the appearance of hyperos- cases, the corneal reflex is often diminished. tosis on CT scanning is distinctive (225–227) (Fig. 30.34). When inner ridge meningiomas compress the cavernous Inner sphenoid ridge meningiomas are treated surgically, sinus, they may interrupt the oculosympathetic pathway and usually from a pterional approach using an operating micro- produce Horner’s syndrome. Pharmacologic testing in such scope and microsurgical instruments (Fig. 30.35). Most of cases shows that the Horner’s syndrome is postganglionic. these tumors cannot be completely removed because of their If an ipsilateral abducens palsy is also present, the cavernous tendency to infiltrate bone, the optic canal, the cavernous sinus is almost always the site of the lesion (219,220). sinus, and the superior orbital fissure (416). Compared with Many patients with inner sphenoid ridge meningiomas patients with meningiomas of the outer sphenoid ridge, pa- that produce diplopia also have proptosis (415). Although tients with inner ridge meningiomas tend to have not only the proptosis may become severe, it is usually minimal and a lower quality of life after surgery but also a shorter postop- associated with a sensation of pressure behind the eye or a erative survival time (177). Sphenoid ridge meningiomas as feeling that the eye or orbit is swollen. The proptosis may a group have the highest risk of recurrence or progression, develop by a variety of mechanisms. The tumor compresses usually within the first 5 to 10 years (178). For this reason, the veins exiting from the orbit and produces venous stasis, postoperative external beam radiotherapy should be per- or the tumor may invade and thicken the bone of the lesser formed for incompletely resected or recurrent tumors to re- (or occasionally the greater) wing of the sphenoid. Finally, duce the rate and extent of progression or recurrence the tumor may extend into the orbit through the superior (242,243,247,280,409,410). orbital fissure. In cases of orbital extension, the optic nerve may eventually become swollen, and visual loss may occur. Tuberculum Sellae (Suprasellar) Meningiomas Most meningiomas that arise from the inner sphenoid ridge produce visual symptoms and signs before they pro- Suprasellar meningiomas represent 3–10% of all menin- duce other neurologic disturbances. Nevertheless, they occa- giomas in most series. Most suprasellar meningiomas origi- sionally extend into the anterior or middle cranial fossa, pro- nate from arachnoid granulations overlying the dura of the ducing symptoms and signs of frontal lobe disease (e.g., tuberculum sellae and its immediate surroundings (Fig. anosmia, disturbances in behavior, seizures) or temporal 30.36). Occasionally, the meningioma has no attachment to lobe disease (e.g., uncinate fits). In the latter instance, there the tuberculum and instead appears to arise from the dia-

Figure 30.34. Neuroimaging of inner sphenoid ridge meningiomas. A, Axial computed tomographic scan after intravenous injection of iodinated contrast material in a patient thought to have thyroid shows marked thickening of the entire greater wing of the right sphenoid, associated with an enhancing soft tissue component compressing the anterior aspect of the right temporal lobe. Note fullness and increased enhancement of the right cavernous sinus, indicating tumor extension into this region. Also note proptosis of the right eye and medial displacement of the right lateral rectus muscle. B, In another patient, T1-weighted magnetic resonance scan, axial view, after intravenous injection of paramagnetic contrast shows a meningioma with soft tissue components on both sides of the sphenoid wing. Note the large intraorbital component and smaller intracranial component. Also note the abnormal enhancement of the pterion. TUMORS OFTHE MENINGES AND RELATED TISSUES 1515

Figure 30.35. Three-dimensional computed tomographic scan after removal of a right sphenoid wing meningioma. Most of the bone representing the greater and lesser wings of the sphenoid has been removed. phragma sellae (417–420). These latter tumors, often called Although such headaches are often frontal, they may occur diaphragmatic meningiomas, are usually located primarily in in any location, and their characteristics are not specific for the retrochiasmatic region rather than being prechiasmatic, tuberculum sellae meningiomas. Other symptoms, typically infrachiasmatic, or suprachiasmatic, as are most of the me- in larger tumors, include mental changes, endocrine dysfunc- ningiomas that arise from the tuberculum sellae. tion, hyposmia, and seizures. Because of the location of the tuberculum sellae with re- The characteristic sign in patients with tuberculum sellae spect to the intracranial portion of the optic nerves and the meningiomas is bilateral optic nerve dysfunction (loss of optic chiasm, painless, progressive visual loss is characteris- vision, color deficits, visual field defects, and optic nerve tic (287,412,421–430). In many cases, the symptoms of vi- pallor). Rare patients have a unilateral optic neuropathy with sual loss are bilateral, whereas in other instances, patients a visual field defect such as a central or paracentral , with the complaint of unilateral visual loss are found to have temporal or nasal hemianopia, altitudinal defect, or general- evidence of bilateral optic neuropathy. ized constriction. Such patients initially may be thought to Nearly half of patients with tuberculum sellae meningio- have a retrobulbar , but progression of the vi- mas complain of headache in addition to visual loss (430). sual loss will eventually challenge this impression. Beware

Figure 30.36. Tuberculum sellae meningiomas. A, A meningioma arising from the tuberculum sellae has extended laterally in both directions to surround and compress both optic nerves and the intracranial portions of both internal carotid arteries. B, Tuberculum sellae meningioma in a 48-year-old woman whose first symptom was reduced vision in the right eye. When she was first examined, the visual acuity in the right eye was decreased and the right optic disc was pale. The patient refused to undergo any neuroradiologic examinations. The vision in her right eye continued to deteriorate, and the eye eventually became totally blind. Subsequently, she began to lose vision in her left eye. She eventually committed suicide. At autopsy, a meningioma of the tuberculum sellae was located between both optic nerves; it extends posteriorly into the anterior angle of the optic chiasm. The right nerve is difficult to recognize because it is flattened and stretched by the tumor (*). The left optic nerve (arrow) is also compressed by the tumor, but the degree of compression is much less. 1516 CLINICAL NEURO-OPHTHALMOLOGY of the diagnosis of chronic optic neuritis! Other patients to the patient’s general physical state rather than to the sur- show evidence of a distal optic nerve syndrome, with loss gery itself (429,430). Complete excision is not always possi- of vision in one eye and a superior temporal field defect ble (178,426,430). As with meningiomas in other locations, in the contralateral, asymptomatic eye (junctional scotoma). recurrence has been described (66,259,260). In patients who Asymmetric damage is common (429,431). have suprasellar meningiomas that are either considered in- In some cases, there is binasal visual field loss from dam- operable or are known to have been subtotally excised, exter- age to the temporal fibers of the intracranial portions of both nal beam radiotherapy to the tumor site may stabilize or even optic nerves. This occurs most frequently in patients with improve visual function (409,442). postfixed optic chiasms. In such cases, the tumor grows be- The visual prognosis in patients with tuberculum sellae tween the two nerves and pushes them laterally against the meningiomas is variable. Most series report an improvement internal carotid arteries (291). More commonly, the field in visual function in 40–70% of cases (422–424,429,443). defects are bitemporal from damage to the optic chiasm. In addition, 50% of eyes had improvement in visual fields When the tumor grows posteriorly or the optic chiasm is or maintenance of normal visual fields (412). Nevertheless, markedly prefixed, the primary field defect may be a homon- most investigators described worsening of visual function ymous hemianopia from damage to the optic tract. A pure in 12–15% of patients (422–424,429,443). optic tract syndrome rarely occurs in this setting. The three most important factors affecting postoperative Many patients with visual loss have associated optic atro- vision appear to be duration of visual symptoms, tumor size, phy, but in some cases, the optic nerves appear normal or and preoperative visual function (8,284,424,429,443,444). there may be subtle atrophy of the peripapillary nerve fiber The earlier the diagnosis is made (and thus the shorter the layer. It is in this latter group of patients that visual loss is duration of visual symptoms), the more likely it is that preop- often mistakenly ascribed to disturbances in the ocular media erative visual function will be relatively good, and tumor (e.g., early ) or to disease of the retina (e.g., macular size will be small. degeneration) (432). This leads to delayed diagnosis that in turn results in a worse visual prognosis. Rarely, tuberculum sellae meningiomas grow so large that Ventricular (Lateral and Third) Meningiomas they produce an intracranial mass effect (430). Papilledema These tumors arise from the tela choroidea in the roof of may develop in this setting and be accompanied by pyrami- the ventricles. As with meningiomas of the fourth ventricle, dal signs or an altered mental status. Some patients with meningiomas of the lateral and third ventricles are quite rare. tuberculum sellae meningiomas complain of diplopia. The Meningiomas of the lateral ventricle account for 0.75 diplopia usually is caused by unilateral or bilateral abducens –4.4% of meningiomas (177,445). They usually become nerve paresis from increased ICP or tumor extension into symptomatic in the fourth decade of life, but they can pro- the cavernous sinus. duce symptoms in childhood (446,447). They are said to Most of the symptoms and signs that occur in patients with tuberculum sellae meningiomas develop over a long occur more often on the left side than on the right (448–450). period of time, usually months to years, but rare patients These tumors usually arise in the area of the trigone, grow- experience sudden visual difficulties (433). ing slowly and expanding initially into the body and ulti- MR imaging is the preferred diagnostic procedure (Fig. mately into the inferior or posterior horn of the ventricle 30.37), although the diagnosis is usually also possible using (Fig. 30.38). Rarely, they extend into the anterior horn or CT scanning (10,225–227,434,435). The suprasellar epicen- project into the via the foramen of Monro. ter, tapered dural base, and bright enhancement with gadolin- The majority of lateral ventricle meningiomas do not ium allow differentiation from a in most produce characteristic or localizing symptoms or signs cases. Cerebral angiography or MR angiography may be (446). Headache, , , and transient visual needed not only to exclude an aneurysm but also to deter- loss caused by increased ICP are the most common present- mine the precise relationship of the tumor to the intracranial ing complaints. Patients often have papilledema. Other vessels. A tumor blush is characteristic of a meningioma but patients initially develop seizures or changes in personality, also occurs in pituitary adenomas. The origin of the feeding mentation, or both (445–447,451). Occasionally, the symp- vessels may differentiate a tuberculum sellae tumor from a toms are episodic, as in the patient described by Wang meningioma originating from the diaphragma sella (poste- et al. (452). rior ethmoidal and ophthalmic artery branches, respec- In addition, about 50% of patients have localizing signs tively). Tuberculum sellae meningiomas may displace or (8,445). There may be a contralateral homonymous hemia- completely surround the internal carotid, ophthalmic, ante- nopia, incongruous and denser below, often with macular rior cerebral, or anterior communicating arteries, making sparing. The field defect is a result of pressure on the optic surgical removal extremely difficult. radiations in the parietal lobe. In some patients, both with The optimal treatment of tuberculum sellae meningiomas, and without hemianopia, there are visual hallucinations, usu- regardless of size, is surgical removal. A pterional approach ally unformed and consisting of flashing lights or jagged is used most often; however, if most of the tumor is within lines, mimicking . A contralateral hemiparesis and the sella turcica (diaphragmatic meningioma), a transsphe- hemisensory loss may be present. In rare cases, cerebellar noidal approach may be used (417,429,430,436–441). The signs—particularly ataxia and nystagmus—are produced by operative mortality is 5–10% and appears to be more related compression of the superior cerebellar peduncle by the TUMORS OFTHE MENINGES AND RELATED TISSUES 1517

Figure 30.37. Neuroimaging appearance of tuberculum sellae meningiomas. A, T1- weighted axial magnetic resonance (MR) image after intravenous injection of paramagnetic contrast shows a small enhancing mass surrounding the intracranial portion of the left optic nerve. B, T1-weighted enhanced sagittal MR image shows the origin of the mass from the tuberculum sellae. C, Computed tomography (CT) scan, coronal view, in another patient shows a larger tumor arising from the tuberculum sellae. D, CT scan, axial view, in the same case as C shows marked suprasellar extension of the tumor. E, Massive extension of a tuberculum sellae meningioma seen on an enhanced T1-weighted MR image, coronal view. tumor. Ocular motor nerve palsies and other cranial neuropa- usual mode of therapy. Patients with hydrocephalus may thies occur infrequently, although abducens paresis may require a ventricular shunt before definitive removal of the occur because of increased ICP. tumor is attempted (446,447,453). Most surgeons advocate Intraventricular meningiomas can usually be identified a sagittal or oblique parieto-occipital cortical approach to easily with CT scanning or enhanced MR imaging avoid damage to motor, speech, and visual areas of the cor- (10,225–227). Once the diagnosis is made, surgery is the tex. In some cases, the tumor can be completely removed, 1518 CLINICAL NEURO-OPHTHALMOLOGY

Figure 30.38. Meningiomas originating in the lateral ventricle. A, A 63-year-old man had a 2-year history of increasing weakness of the right side of the body, difficulty with speech, and inability to read. He was eventually found to have a right hemiparesis with involvement of the face and a right homonymous hemianopia. A meningioma (arrow) had originated in the choroid plexus of the left lateral ventricle and had extended into cerebral tissue (arrowhead) to damage the internal capsule and optic radiations, producing the patient’s neurologic symptoms and signs. B, A large, right, intraventricular meningioma in a patient with a left homonymous hemianopia. The tumor has destroyed the right lateral geniculate body. The left lateral geniculate body is intact (arrowhead). (A, From Lindenberg R, Walsh FB, Sacks JG. Neuropathology of Vision: An Atlas. Philadelphia: Lea & Febiger, 1973.) whereas in others, the degree of cerebral invasion is such that ally unrelated to head or body position—is the major early total removal is impossible. In the latter cases, postoperative symptom, but loss of vision or diplopia may also occur. external beam radiation therapy may be beneficial. Regard- Confusion, lethargy, disturbance of gait, and urinary inconti- less of the therapy used, the eventual outcome in patients nence may develop. Papilledema is the rule. When the tumor with meningiomas of the lateral ventricle appears to be quite is primarily posterior, there may be pressure on the midbrain good, with many of these patients being able to function that produces a partial or complete dorsal mesencephalic normally for many years (445–447). (Parinaud’s) syndrome. Meningiomas of the third ventricle are even less common Meningiomas located in the third ventricle can usually be than meningiomas in the lateral ventricle. In some instances, removed with relative ease with the aid of the operating the tumor arises in the lateral ventricle and extends into the microscope through a transcallosal approach via the foramen third ventricle through the foramen of Monro. In rare cases, of Monro, whether by splitting the fornices or using a sub- often in children, the meningioma arises from the tela choroi- ependymal approach. The risk to memory with this approach dea of the third ventricle itself (72,447,454–462). is significant, particularly if the tumor is large. Most patients The clinical characteristics are nonspecific and are related have little residual neurologic deficit following surgery to the effects of increased ICP. Intermittent headache—usu- (463).

SARCOMAS OF THE MENINGES AND BRAIN A variety of sarcomas occur within the cranial cavity. usually at the base of the skull, but also in other locations These include chondrosarcomas, osteogenic sarcomas, pri- (251,465–481). In fact, chondrosarcomas represent only mary sarcomas, and rhabdomyosarcomas (embryonal sar- 0.15% of all intracranial tumors and 6% of lesions at the comas). The tumor previously known as reticulum cell sar- base of the skull (482). coma arises from cells of the mononuclear-phagocytic Intracranial chondrosarcomas arise not only within the (reticuloendothelial) system and is, in fact, a form of diffuse, bone of the skull but also occasionally within the dura, pre- large cell lymphoma. sumably from the meningeal fibroblasts that line the under- surface of the dura and arachnoid (21,483,484). Such tumors CHONDROSARCOMA present as intracranial masses without bony involvement. Their neuroimaging appearance may mimic that of a menin- This rare tumor arises from cartilaginous rests in or near gioma. bone. Although the age range is broad, it usually occurs in Many but not all intracranial chondrosarcomas have a spe- adults between 25 and 50 years of age (464). Males are cific histologic appearance. They are composed of focal primarily affected, with a male-to-female ratio of about three areas of undifferentiated mesenchymal cells surrounded by to one. Although chondrosarcomas most commonly occur areas of mature and immature hyaline cartilage with myxo- in the extremities, they may occasionally arise intracranially, matous foci of atypical or immature chondrocytes (485). TUMORS OFTHE MENINGES AND RELATED TISSUES 1519

Many of these tumors are highly vascular and contain areas OSTEOGENIC SARCOMA of ossification and dysplastic bone. Such variants are called Osteogenic sarcoma is a highly malignant tumor charac- mesenchymal chondrosarcomas and are thought by some terized by a sarcomatous fibroblastic stroma in which osteo- authors to be a more malignant form of chondrosarcoma blastic activity has induced the formation of tumor osteoid with a greater tendency for recurrence and metastasis (481). and bone (497). This definition excludes fibrosarcomas and When intracranial chondrosarcomas arise at the base of chondrosarcomas that arise in bone but do not produce either the skull, they commonly produce isolated cranial neuropa- matrix or bone structure. thies, usually abducens nerve palsy, that have an insidious Osteogenic sarcoma is the most common malignancy of onset and a slowly progressive course (469–475,486). They bone. It usually occurs in the long tubular bones of the ex- may invade the cavernous sinus, producing a painful or occa- tremities in young adults. Only rarely is this tumor encoun- sionally painless ophthalmoplegia (472,487,488). They must tered in patients over 40 years of age, and in most of these be differentiated from nasopharyngeal carcinomas, chordo- cases, the tumor is associated with pre-existing Paget’s dis- mas, and other tumors that occur at the base of the skull ease. Men are affected slightly more often than women. (221). When these tumors arise in or near the sphenoid ridge, Despite its common occurrence in the bones of the extrem- tuberculum sellae, sella turcica, or paranasal sinuses, they ities, primary osteogenic sarcoma is one of the rarest tumors may invade the orbit, producing progressive proptosis asso- occurring in the skull (498). This lesion usually arises from ciated with ophthalmoplegia and visual loss (475,489). Tu- the bones covering the cranial vault; however, it may also mors that occur in the posterior ethmoid and sphenoid si- occur at the base of the brain (499). Patients with this tumor nuses may erode the bone of the optic canal, producing loss may present with diplopia, visual loss, or both. of vision from compression of the intracanalicular portion As with other forms of intracranial sarcoma, osteogenic of the optic nerve. The visual loss may be gradual or sudden sarcomas occasionally develop following cranial irradiation (490,491). In the latter instance, the patient initially may be (500,501). thought to have retrobulbar optic neuritis. Chondrosarcomas in this region also produce progressive visual loss, ophthal- PRIMARY SARCOMA OFTHE MENINGES AND moplegia, or both through their effects on the intracranial BRAIN portions of the anterior and the ocular motor nerves (467,474,492,493). According to Russell and Rubinstein, primary intracranial As noted above, not all intracranial chondrosarcomas arise sarcomas can be distinguished from other sarcomas and from meningiomas with malignant histologic changes (21). These at the base of the brain. Some of these lesions present as sarcomas occur predominantly in infants and children and masses in the frontal, temporal, parietal, or occipital regions. are quite rare (502). The clinical history is short, and deterio- Such tumors produce symptoms and signs of increased ICP ration is usually rapid, either because of the intractable be- as well as localizing symptoms and signs from compression havior of the tumor or because of its rapid recurrence after of surrounding brain tissue. surgical removal. Because some intracranial chondrosarcomas arise from Some primary intracranial sarcomas are clearly of dural the meninges without involvement of adjacent bone, it is not or leptomeningeal origin and only secondarily involve the surprising that chondrosarcomas occasionally occur within substance of the brain. Others arise within the brain and the . remain below its surface. Such tumors probably originate Most chondrosarcomas produce neurologic symptoms from the pial sheaths that enclose the perforating blood ves- and signs either by their mass effect or by compression or sels or from the tela choroidea of the ventricles. invasion of surrounding tissue (481). Distant metastases, he- The presenting symptoms and signs of patients with pri- matogenous seeding, and lymphatic spread are all un- mary intracranial sarcomas depend on the site of origin of the common. tumor and the rapidity with which it grows. Many patients The most effective treatment is wide surgical excision. initially present with symptoms and signs of increased ICP With the improvement in microsurgical techniques, com- (503). Others develop seizures, hemiparesis, hemisensory plete removal of these tumors, at least those at the base of the deficits, cerebellar signs, or cranial neuropathies. In most skull, is often possible (471,472,474,475,482). When total cases, the development of multiple symptoms and signs is removal is not possible, proton-beam radiation therapy or rapid. stereotactic radiosurgery may be helpful in achieving local The appearance of a primary intracranial sarcoma is not control (469,474,488,494). distinctive. The tumor often forms a fleshy soft mass whose The prognosis of intracranial chondrosarcoma correlates borders are irregular and often ill defined. Because of rapid with histologic grading, which in turn depends upon the de- growth, secondary changes such as necrosis, hemorrhage, gree of cellular atypia and mitoses (495,496). Patients with and cystic degeneration can frequently be observed on the the most benign form of the tumor have a 5-year survival cut surface of the tumor. In rare cases, the mass is well rate of about 90% and a 10-year survival rate of 83%, circumscribed and can be removed surgically with good re- whereas patients with the most malignant form have 5- and sults (503,504). In other cases, however, there is extensive 10-year survival rates of 43% and 29%, respectively. The and diffuse distribution of tumor: primary diffuse sarcomato- rate of metastasis also varies according to grade. Chondro- sis of the meninges (505) (Fig. 30.39). sarcomas may recur up to 8 years after resection. Primary intracranial sarcomas occasionally disseminate 1520 CLINICAL NEURO-OPHTHALMOLOGY

Figure 30.39. Primary intracranial polymorphic cell sarcoma. A, Tumor compresses the ventral surface of the left side of the mesencephalon. B, Photomicrograph of the left side of the ventral surface of the mesencephalon shows that tumor cells surround the fibers of the left oculomotor nerve as they exit from the brain stem. within the CNS (21,503,506,507). Primary intracranial sar- signs by direct invasion of the meninges from the nasophar- comas, particularly fibrosarcomas, may occur after cranial ynx, paranasal sinuses, middle ear, and orbit (519,530–536). irradiation as well as spontaneously (508–511). Most of Such tumors invade the skull base or extend through neural these lesions occur after radiation therapy for pituitary ade- foramina. When they encounter a cranial nerve, they spread nomas (511–513). The latent period between radiation and via the endoneural and perineural spaces (537,538). They the discovery of the sarcoma usually ranges from 5–12 years may produce progressive diplopia from damage to the ocular but may be decades (511,514,515). motor nerves with or without facial hypesthesia or dysesthe- sia from involvement of the trigeminal nerve (519,539–541). RHABDOMYOSARCOMA The facial nerve is often affected, particularly by lesions that originate in the middle ear (519,531). Such lesions may also Rhabdomyosarcomas are malignant tumors composed of produce drainage from the middle ear, and the patients may striated muscle cells in patterns suggestive of neoplastic ana- at first be thought to have simple otitis media. In some cases, logs of various stages of normal muscle development. Four rhabdomyosarcomas that invade the skull base compress the histologic forms occur: embryonal, alveolar, pleomorphic, brain stem and cerebellum. In some instances, extracranial and botryoid (516,517). Rhabdomyosarcoma is the most common soft tissue sar- rhabdomyosarcoma may metastasize to the CNS (506,542). coma that occurs in infants and young children (517–519). Rarely, rhabdomyosarcoma originates within the CNS There is no gender predilection (519). Between 30% and (541,543–549). In such cases, it occurs as a mass lesion, 50% of rhabdomyosarcomas arise in the head and neck producing increased ICP, focal neurologic signs, or both. (516,517,520). Most ocular abnormalities in patients with These tumors may arise within the cerebral hemispheres, rhabdomyosarcoma occur when the tumor originates in the within the cerebellum, or even within one of the ventricles. orbit or metastasizes to it (521–528). Such patients develop The origin of primary intracranial rhabdomyosarcoma is rapid, progressive proptosis, ophthalmoplegia, and blindness thought to be from multipotential mesenchyme derived from if appropriate therapy is not immediately initiated. Rhabdo- the neural crest. myosarcomas that arise in the paranasal sinuses or nasophar- In patients with known peripheral rhabdomyosarcoma ynx may invade the orbit. In most cases, such invasion pro- who develop progressive cranial neuropathies, the diagnosis duces obvious evidence of orbital disease, but in other of meningeal invasion is usually suspected and may be con- instances, there is rapid visual loss that may initially be mis- firmed by biopsy. Neurologic symptoms and signs may de- taken for retrobulbar neuritis (529). velop from metastasis (particularly when the primary tumor Rhabdomyosarcomas may affect the CNS by several has not yet been discovered) or from a primary rhabdomyo- mechanisms. About 15% of rhabdomyosarcomas that origi- sarcoma of the CNS. Unfortunately, the diagnosis may not nate in the head and neck produce neurologic symptoms and be made until a biopsy is performed. TUMORS OFTHE MENINGES AND RELATED TISSUES 1521

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