Meningiomas

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Chapter 36 Meningiomas

Clinical Pearls

Meningiomas are believed to derive from the arachnoid cap cells around arachnoid granulations near venous sinuses, cisterns, ventricles, and brain. They can be found anywhere there is known pia, arachnoid, or dura. These tumors exhibit a wide variety of behaviors from benign to extremely aggressive. The etiology of meningiomas is unclear but some are associated with genetic aberrations such as partial loss of chromosome 22, prior trauma, and radiation therapy.

The latest World Health Organization (WHO) grading system of meningiomas evaluates these neoplasms from grades I to III. Grade I meningiomas have nine subtypes ranging from fibroblastic to psammomatous. Despite the different histological patterns there is no prognostic significance among the subtypes of grade I meningioma. Grade II represents an atypical meningioma and implies the presence of mitosis, or three or more of such features as increased cellularity, brain invasion, or necrosis. Grade III anaplastic malignant meningiomas are characterized by highly active mitosis, and their tumor cells resemble carcinoma or sarcoma. Metastases are rare in meningioma but can occur in the lungs, liver, bone, and heart.

The treatment of meningiomas depends on a variety of factors such as their growth rate, radiological characteristics, location, and patient clinical status and age. The advent of magnetic resonance imaging (MRI) has brought the age of more incidentally diagnosed lesions. The natural history of meningiomas varies, as do the growth rates. Some incidentally discovered meningiomas remain stable and can be observed, especially in elderly patients with few symptoms or signs. Meningiomas are symptomatic in a wide range of patient ages and locations and thus warrant excision. Total surgical excision of meningiomas is the treatment of choice.

A wide spectrum of surgical approaches can be employed to radically excise a meningioma. Preoperative embolization can decrease intraoperative blood loss in selected patients. Postoperative radiation therapy, radiosurgery, and hormonal therapy is required for incompletely resected lesions or those with malignant characteristics.

Simpson classification of meningioma provides a general estimate of recurrence after resection. The resection ranges from grade I resection, which is complete removal, to grade IV subtotal resection, and grade V, which is decompression of the tumor. The recurrence rates are as low as 9% for grade I resections and as high as 40% for grade IV resections.

In 1922, Harvey Cushing presented a series of 85 meningeal tumors in his Cavendish lecture and coined the term meningioma to describe these lesions.1 Years later with Louise Eisenhardt, he created a definitive monograph on these tumors.2 He believed that all meningeal tumors arose from the arachnoidal cap cells that are particularly abundant in the arachnoid granulations.

Meningiomas are the most common brain tumor and have a wide variety of clinical behaviors. Although most of them behave rather nicely, there are some that are extremely aggressive. Little is known about the reasons for this difference in natural history. The etiology of meningiomas remains unknown, but previous radiation therapy and monosomy or partial loss of chromosome 22 are important factors. Radiation therapy, at a high or low dosage, can cause meningiomas even after several years of treatment.3

Epidemiology

Meningiomas constitute 15% to 20% of all primary intracranial tumors in surgical series, but their incidence in routine screening is 1 in 100 population. Their incidence increases with advancing age.4 They predominantly affect women with an overall male-female ratio of 1:2.5. This difference is increased for intraspinal meningiomas with a ratio of 1:10 in comparison with an intracranial ratio of 2:3. Meningeal tumors are rare in children but tend to be more aggressive when they occur in children. They represent 0.4% to 4.1% of all childhood brain tumors and constitute 1.5% to 1.8% of all meningiomas.5,6 Pediatric meningiomas tend to be more frequent in males with a male-female ratio of 1.2 to 1.9:1 and have a higher incidence of ventricular location.7,8

In one study, symptomatic meningiomas were encountered in 2.0 per 100,000 of the population and asymptomatic ones in 5.7 per 100,000, with an overall incidence of 7.7 per 100,000.9

Imaging

Contrast-enhanced magnetic resonance imaging (MRI) scans with the addition of arterial and venous sequences are the most important studies to evaluate these tumors. Computed tomography (CT) may provide valuable information about bony anatomy. Three-dimensional (3D) MRI or CT scans provide useful information about tumor features and surrounding anatomy and are particularly useful for surgical planning.

Plain radiographs may demonstrate hyperostosis, irregular cortex, erosion, tumor calcifications, and enlargement of vascular grooves (middle meningeal artery).

Computed Tomography

Meningiomas on nonenhanced CT usually appear as well-circumscribed extra-axial lesions, hyperdense (70-75%), isodense (25%), or hypointense (1-5%) to adjacent parenchyma.8 Calcifications ranging from microscopic psammoma bodies to dense sclerosis are found in 25% of patients. Necrosis, cysts, and hemorrhage are seen occasionally (8-23%).8,10 With contrast agent, they usually enhance brightly.

Magnetic Resonance Imaging

T1-weighted imaging shows meningiomas as isointense or moderately hypointense to gray matter lesions. Calcifications and highly fibrous areas are hypointense. FLAIR (fluid-attenuated inversion recovery) is helpful to demonstrate edema, seen as a hyperintense signal in the adjacent parenchyma. T2-weighted imaging may present a wide range of possible signal intensities. Usually isointense or mildly hyperintense, it can show hypointensity if the meningioma is calcified or highly fibrous. Massive surrounding edema is seen as a hyperintense signal. Arterial feeders to tumor are seen as arborizing flow voids (hypointense). Pial blood vessels present as surface flow voids between tumor and parenchyma.11 T2-weighted gradient echo (GRE) may “bloom” as parenchymal low signal, suggesting calcifications or intratumoral microhemorrhages.

T1 contrast enhancement shows meningiomas as heterogeneous clearly defined hyperintensive images. The “dural tail” may enhance in 35% to 80%, but is not specific.11,12 Magnetic resonance (MR) angiography and venography are noninvasive options to demonstrate tumor blood supply, vascularization, drainage veins, and sinus compromise.

Functional MRI is based on increased brain hemodynamics in response to cortical neuronal activity due to certain stimulus performed during imaging. It can be helpful in surgical planning for localization of motor, sensory, and language regions. Diffusion may differentiate benign from atypical or malignant meningiomas.13,14 Perfusion reveals differentials in relative cerebral blood volume, allows us to distinguish meningiomas from dural metastases,15 and according to some authors also discerns typical from atypical histological grades.8,16 Spectroscopy shows high choline peak, low or absent N-acetylaspartate (NAA) and creatinine levels, and variable amounts of lactate. Some of them also present high alanine and glutamate/glutamine levels on MR spectroscopy.17

Pathology

Hormone Receptors

Meningioma growth may be related to hormonal status due to the presence of estrogen and progesterone receptors. The tumor may become clinically evident during pregnancy or in the luteal phase of the menstrual cycle.23 The expression of progesterone receptors alone in a meningioma could be related to a favorable behavior. Absence of both progesterone and estrogen receptors or the presence of estrogen receptors alone correlates with aggressive clinical behavior, progression, and recurrence after complete surgical resection.24 Despite the presence of these receptors, drug therapies targeting hormonal status have not been particularly successful.

Classification

Histological subtypes are classified according to the most recent WHO grading system published in 200725 (Box 36.1). In the 2000 WHO classification of meningiomas, brain invasion was associated with aggressive behavior and increased probability of recurrence, but was not included as a diagnostic criterion for grade II or grade III tumor.3 Perry and associates demonstrated that brain invasion indicated a greater likelihood of recurrence and felt that it should be considered one of the diagnostic features of grade II meningioma.26 Following these findings, brain invasion by a meningioma is now an independent criterion for WHO grade II. Subtype classification does not appear to influence prognosis unless atypia or malignancy is evident.

Decision Making

The treatment of meningiomas depends on their natural growth rate, radiological characteristics, and location; the patient’s clinical status; and an assessment balancing the potential morbidity of conservative versus invasive treatment. With the advent of better imaging techniques, more meningiomas are being incidentally discovered. Issues in decision making concerning proper management become particularly important.

In order to integrate these variables some treatment algorithms have been developed. Dr. Takeshi Kawase and his group (Adachi and associates30) in 2006 presented a set of rules for treating cranial base meningiomas. They give a score to each tumor based on predetermined risk characteristics:

A higher score number (risk factor) implies a lower chance of complete resection.

Dr. Joung Lee31 and his group at the Cleveland Clinic designed the “CLASS” algorithm for the treatment of all meningiomas. This algorithm compares negative features (comorbidity, location, and age) against benefits (size and symptoms) and assigns a score:

Patients with a score of +1 or higher had a 1.9% rate of poor outcome; those with a score of 0 to 1 had a 4% rate of poor outcome; and of those with a score of −2 or less, 15% had a poor outcome. Therefore, more negative features are related to an increased chance of having an undesirable postoperative outcome.

Even with these grading systems, the final management strategy for meningiomas should be based on the patient’s age, general medical condition, and wishes for treatment.

Conservative Treatment

Around two thirds of asymptomatic meningiomas do not continue to grow and may be observed at appropriate time intervals. Absolute growth rates of meningiomas vary between 0.03 and 2.62 cm3 per year. Several studies following the behavior of asymptomatic meningiomas showed minimal growth during the follow-up period. In a retrospective study of tumor growth rate in 37 patients, 9 of the 37 (24.3%) showed tumor growth during a mean follow-up period of 4.2 years. Annual growth rates were calculated as the difference in tumor volume between the initial and latest imaging, divided by the time interval (years) between these determinations, with tumor growth defined as an annual increase in tumor volume more 1 cm3 per year. In this study they associate the age of patients and the volume of the tumor at its initial diagnosis with growth rate increased. They concluded that young patients and those with large tumors should be carefully observed.32 Nakamura and colleagues33 studied 41 patients with asymptomatic meningiomas, reporting a majority (66%) of growth rates less than 1 cm3 per year. They also correlated growth rate with patient age but did not consider initial tumor size as a predictive factor for tumor growth. Yano and Kuratsu in their study of surgical indications for asymptomatic meningiomas reported 37% of tumor growth during a period of observation of 3.9 years and only 6.4% becoming symptomatic.34 Some authors recommend the surgical resection of meningiomas when the tumor growth rate is greater than 1 cm3 per year.32,33 Radiological features such as partial or complete calcification is related to slow growth rate or absence of it, so these tumors may be kept only under observation. Meningiomas that remain asymptomatic but show displacement and compression of delicate structures as spinal cord, optic nerve, chiasm, and brainstem, or with considerable surrounding edema, should be considered for early treatment. Observation alone, with periodic neurological and MRI evaluation follow-up, first at 3 months, second at 6 months, and then every year, is reasonable for asymptomatic or minimal symptomatic elderly patients with fewer than 10 to 15 years of remaining life expectancy.

Surgical Treatment

General Surgical Planning

General Recurrence Rate

In 1957 Simpson35 classified meningioma resection as follows: grade I, complete removal, including resection of dura and bone; grade II, complete tumor removal with coagulation of dural attachment; grade III, complete tumor removal without resection or coagulation of dural attachments; grade IV, subtotal removal; and grade V, decompression. This classification remains useful for evaluating recurrences. In Simpson’s series, grade I through grade IV tumors had recurrence rates of 9%, 19%, 29%, and 40%, respectively, at a follow-up period of 10 years.

Considerations by Location

Convexity Meningiomas

Convexity meningiomas arise from any part of the cranial convexity without involving dural sinuses. They are frequently located around the coronal suture and frontotemporal junction. They can be totally removed including involved dura and bone with great chances of cure. They represent between 15% and 19% of all meningiomas.

Surgical Technique

Operative Results

In a series of convexity meningiomas Black and co-workers36 reported no surgical fatality and no significant difference in morbidity between age groups younger and older than 65 years. The overall morbidity rate was 5.5%. The 5- and 10-year survival rate was 90% with overall recurrence rate of 4.3%. The 5-year recurrence rate for WHO grade I tumors was zero, for grade II 27.2%, and for grade III was 50%. In their series 15 patients (9%) underwent radiation therapy.

Parasagittal Meningiomas

These meningiomas represent about 16.8% of meningiomas and are classified in relation to their location along the superior sagittal sinus (SSS) and its invasion. The anterior third extends from the crista galli to the coronal suture, the middle third from the coronal to the lambdoid sutures, and the posterior third from the lambdoid suture to the torcular. Sindou and associates have classified sinus involvement as follows:

To achieve a Simpson grade I or II radical resection, the infiltrated SSS should be removed with the tumor. They represent about 16.8% to 25.6% of all intracranial meningiomas.38

Surgical Technique

Operative Results

In Black and co-workers’36 series the anterior third of the SSS was involved in 12.8% of tumors, the middle third in 69.2%, and the posterior third in 17.9%. In 63.2% of patients there was total tumor resection, Simpson grades I and II. In 14 patients (36.8%) residual tumor was found on postoperative imaging, and 13.2% of those had tumor progression. Recurrence-free survival rate was 94.7% at 5 years.

Falx Meningiomas

These meningiomas arise from the falx cerebri and tend to grow and compress the medial surface of the cerebral hemispheres. They can be classified according to involvement of the falx in longitudeal dimension. Like parasagittal meningiomas, they can be divided into anterior, middle, or posterior types. The classification proposed by Yasargil40 separated them into outer falx meningiomas, which arise from the body of the falx, and inner falx meningiomas that arise adjacent to the inferior sagittal sinus (ISS). Falcine meningiomas represent 8.5% of all intracranial meningiomas.41

Evaluation

About 60% of falx meningiomas present the dural tail sign.41 MRVA or angiographies are useful to determine the displacement or involvement of the anterior cerebral artery (ACA). The venous phase shows the SSS of ISS invasion and the localization of venous drainage.

Surgical Technique

Operative Results

In the series of 68 patients presented by Chung and colleagues,41 85.2% had total resection with no evident recurrence and 92.6% achieved a good outcome (no neurological deficit or complications). SRS was performed as a postoperative adjunctive treatment in six patients.

Olfactory Groove Meningiomas

Olfactory groove meningiomas (OGM) arise in the midline of the anterior fossa, from the apophysis crista galli to the planum sphenoidal. Usually bilateral, they grow over the cribriform plate and frontosphenoidal suture. The invasion of the ethmoid bone and the paranasal sinuses makes a complete resection difficult and increases the chance for recurrence. Small meningiomas displace laterally the olfactory nerves and large tumors push them together with the optic chiasm into a posteroinferior direction. Blood supply of this tumor usually comes from ethmoidal branches of the ophthalmic artery, the anterior branch of the middle meningeal artery (MMA), and meningeal branches of the internal carotid artery (ICA). They represent 10% of all intracranial meningiomas.

Surgical Technique

The frontotemporal (pterional) approach is preferred for small and medium-sized tumors and bifrontal craniotomy is recommended for large lesions.

Approach

In the frontotemporal (pterional) approach, the skin incision is begun 1 cm anterior to the tragus at the level of the zygomatic arch and is extended superiorly, then curving anteriorly from the superior temporal line to the midline, just behind the hairline, and extended behind. The scalp flap is reflected anteriorly with sharp dissection against the galea. The superficial and deep fascia of the temporalis muscle are incised 1 cm posterior and parallel to the course of the frontal branches of the facial nerve and retracted anteriorly with the skin flap. The temporalis muscle is incised posterior to the superficial temporal artery and lifted anteriorly and inferiorly using fish hooks to expose the roof and the lateral rim of the orbit. At the supraorbital ridge the supraorbital nerve and vessel run through the supraorbital foramen, and care must be taken to preserve them. The pericranium is dissected behind and incised as posteriorly as possible and then is reflected anteriorly over the scalp flap. A keyhole is placed behind the suture between the frontal bone and the frontal process of the zygomatic bone. Bur holes are made in the floor of the middle fossa and if it is necessary posterior to the superior orbital rim. The bone flap is done with a craniotome. The superior arch of the orbit may be removed for a wide exposure in large tumors. In the bifrontal approach the skin is cut posteriorly to the frontal hairline from zygoma to zygoma. The craniotomy is performed through bur holes placed on each side of the SSS with a high-speed drill. Approximately 1 cm of bone is left posteriorly to the orbital rim.

Microsurgical Resection

For the frontotemporal approach the dura is opened in a C-shape fashion with an anterior base along the sphenoid ridge. It is folded and anchored with sutures. Under magnification the sylvian fissure is dissected allowing the visualization and opening of the optic carotid and carotid oculomotor cisterns. The arachnoid membrane between the optic nerve and frontal lobe is incised and opened to allow the retraction of the frontal lobe and dissection from the tumor capsule. The internal debulking is performed with an ultrasonic aspirator or laser. Following this step the tumor capsule can be easily disected from the underlying brain. The blood supply is occluded along the base and the tumor capsule. With microdissection the tumor is removed. The dura attachment in the anterior fossa base is coagulated, the crista galli cuted and drilled. After hemostasis control the dural defect is closed in a watertight fashion with the vascularized pericranial flap, avoiding tension on the brain and allowing tenting to the bone flap to occlude dead space. If the frontal sinus is exposed it must be cranialized by removing its posterior wall. The frontonasal ostia are occluded with muscle and bone.

For the bifrontal approach the dura is opened in both sides of the SSS. The sinus is ligated or sutured and divided on its anterior third. The falx cerebri is cuted and with gentle retraction the tumor is exposed. Microsurgical tumor removal steps are followed as usual. The posterior surface of the tumor can be closely related to the ACA; care must be taken to avoid its injury. The frontotemporal artery is frequently attached to or encased in the tumor and should be released but can be sacrificed without consequence. After the tumor removal, the involved dura must be resected and coagulated. Hyperostotic bone should be drilled until normal bone is identified assessing the ethmoidal sinus in order to remove all invaded cavities. The dura defect must be closed in a watertight fashion to avoid CSF leakage.

Tuberculum Sellae Meningiomas

Tuberculum sellae meningiomas (TSMs) arise from the dura of the tuberculum sellae, diaphragma sellae, chiasmatic sulcus, and limbus sphenoidale. Usually bilateral, they grow from the midline over one side. They can invade the suprasellar region as other meningiomas with different dural origins. TSM can be distinguished from OGM by the displacement of the optic nerves and chiasm. TSMs elevate the chiasm and optic nerves superolaterally, but OGMs displace the chiasm downward and posteriorly as they grow. TSMs represent 5% to 10% of all intracranial meningiomas.43

Surgical Technique

Bifrontal, frontotemporal, frontolateral, and the expanded endonasal approaches should be considered for these meningiomas.

Expanded Endonasal Approach

Provide access to the anterior skull base extending from the crista galli to the foramen magnum;44 all 12 cranial nerves and the carotid and vertebrobasilar arteries can be seen through the nose. This approach should be considered only for small TSMs measuring less than 4 cm owing to the limited lateral explosion. Tumors arising lateral to the optic nerve or beyond the midline of the superior orbit are best approached via craniotomy if the objective of surgery is total removal.44 Neuronavigation is commonly used.