As mentioned earlier in the Primer, each type of brain tumor arises from a specific cell type.

The cell of origin for the meningioma is call the arachnoid cap cell, found on the surface coverings (called meninges) of the brain in the paccionian granulations. These serve as the one-way valve system between the water system of the brain and the veins that drain from the brain to the heart. Interestingly, these tumors have an embryologic relationship with cells found in the muscle layer of the utereus. In fact, it is exceedingly difficult for the pathologist to distinguish the meningioma from the fibroid tumors of the utereus under the microscope. Also, they share the characteristic female hormonal receptors (estrogen and progesterone) on their cell surfaces. This characteristic has lead to the testing of anti-estrogen receptor agents, such as tamoxifin, as a growth-inhibiting agent in these tumors. Clinical studies to date have failed to provide siginificantly positive results. Meningiomas are rarely malignant in their behavior. But when malignant, meningiomas grow rapidly and are destructive; they are quite difficult to treat, and recur oftentimes in less than a year after surgical removal. They are also difficult for the pathologist to diagnose under the microscope. Probably the only finding that correlates well with the diagnosis is that of numerous cells seen in division ("mitosis"). The pathologist may occasionally speak of brain and skull invasion, cells with an abnormal appearance, or other bizarre findings, however none of these completey fit the diagnosis. Ultimately, the diagnosis is determined by the activity of the particular tumor over time.

A meningioma is a tumor that arises from the meninges — the membranes that surround your brain and spinal cord. Most meningiomas are noncancerous (benign), though, rarely, a meningioma may be cancerous (malignant). Some meningiomas are classified

A meningioma that causes no significant signs and symptoms may be monitored over time. which reside in the arachnoid layer covering the surface of the brain. The problem of classifying meningioma is that arachnoidal cells may express both mesenchymal and epithelial characteristics. Other mesodermal structures also may give rise to similar tumors (eg. meaning they're neither benign nor malignant. In rare cases. Meningiomas may occur intracranially or within the spinal canal. The current . A meningioma doesn't always require immediate treatment. Meningiomas occur most commonly in older women. including childhood. See the images below. Meningiomas commonly are found at the surface of the brain. meningiomas occur in an intraventricular or intraosseous location. hemangiopericytomas or sarcomas). They are thought to arise from arachnoidal cap cells.as atypical. Case 1: Bone-window CT reveals calcification of the meningioma. either over the convexity or at the skull base. The classification of all of these tumors together is controversial. Case 1: MRI of a meningioma on plaque. but rather something in between. But a meningioma can occur in males and at any age.

rarely. Undoubtedly. signs and symptoms may include: • • • • • • Changes in vision. spine the tumor is situated. advances in molecular biology will allow scientists to determine the exact genomic aberration responsible for each specific neoplasm.trend is to separate unequivocal meningiomas from other less well-defined neoplasms. Symptoms Signs and symptoms of a meningioma typically begin gradually and may be very subtle at first. such as headaches that worsen over time History Meningiomas produce their symptoms by several mechanisms. Seek emergency care if you have: • • Sudden onset of seizures Sudden changes in your vision or memory Make an appointment to see your doctor if you have: • Persistent signs and symptoms that concern you. such as seeing double or blurriness Headaches that worsen with time Hearing loss Memory loss Seizures Weakness in your arms or legs When to see a doctor Most signs and symptoms of a meningioma evolve slowly. They may cause symptoms by irritating the underlying cortex. but sometimes a meningioma requires emergency care. Depending on where in the brain or. compressing the brain or the cranial .

producing hyperostosis[3] and/or invading the overlying soft tissues. Brown-Sequard (hemispinal cord) syndrome Exophthalmos. meningiomas can cause seizures. These stereotypical symptoms are not pathognomonic of meningiomas in these locations. III. urinary incontinence Anosmia with possible ipsilateral optic atrophy and contralateral papilledema (this triad termed Kennedy-Foster syndrome) Multiple cranial nerve deficits (II. • Compression: Localized or nonspecific headaches are common. apathy. dysphasia.nerves. • Stereotypic symptoms: Meningiomas in specific locations may give rise to the stereotyped symptoms listed in the Table. meningiomas in these locations may remain asymptomatic or produce other unlisted symptoms. • Irritation: By irritating the underlying cortex. apathy or disinhibited behavior. leading to decreased vision and diplopia with associated facial numbness Contralateral hemianopsia Decreased hearing with possible facial weakness and facial numbness Cavernous sinus Occipital lobe Cerebellopontine angle Spinal cord Optic nerve Localized spinal pain. often. and/or somnolence. Compression of the underlying brain can give rise to focal or more generalized cerebral dysfunction. or inducing vascular injuries to the brain. Symptoms and Signs Associated with Meningiomas in Specific Locations • Location Parasagittal Subfrontal Olfactory groove Symptoms Monoparesis of the contralateral leg Change in mentation. monocular loss of vision or blindness. IV. monocular optic nerve swelling with optociliary shunt vessels Seizures. multiple cranial nerve palsies if the superior orbital fissure involved May protrude within supratentorial and infratentorial compartments. they may occur with other conditions or lesions. ipsilateral dilated pupil that does not react to direct light stimulation but might contract on consensual light stimulation. Sphenoid wing Tentorial . as evinced by focal weakness. The signs and symptoms secondary to meningiomas may appear or become exacerbated during pregnancy but usually abate or improve in the postpartum period. VI). V. New-onset seizures in adults justify neuroimaging (eg. Table 1. MRI) to exclude the possibility of an intracranial neoplasm. Conversely.

ipsilateral weakness. positive Hoffman sign. Meningiomas of the skull base may narrow and even occlude important cerebral arteries. o Compression of the dominant (usually left) parietal lobe may give rise to Gerstmann syndrome: agraphia. • Spinal meningiomas may give rise to a Brown-Sequard syndrome (ie. ultimately. decrease in position sense). • Involvement of the cranial nerves may lead to anosmia. facial paresis. acalculia. ultimately. involvement of cranial nerves. and finger agnosia. depending on their location. namely Castleman syndrome. However. Causes • Trauma and viruses have been investigated as possible causative agents for development of meningiomas. sphincteric weakness and. Physical symptoms The physical findings mirror the aforementioned symptoms and include signs due to raised intracranial pressure. tongue atrophy associated with fasciculation • Vascular: This presentation. • Compression of the underlying parenchyma may give rise to pyramidal signs that are exemplified by pronator drift. possibly presenting either as transient ischemic attack (TIA)–like episodes or as stroke. o Compression of the occipital lobes leads to a congruent homonymous hemianopsia. should be considered. no definitive proof has yet been found. visual field defects. o Meningiomas in the vicinity of the sella turcica may produce panhypopituitarism. • Raised intracranial pressure leads to papilledema. o Rarely. complete quadriparesis or paraparesis. optic atrophy. decreased mentation and. hyperreflexia. sphincteric troubles.producing symptoms by compressing specific structures within these 2 compartments[4] Foramen magnum Paraparesis. diplopia. Parietal-lobe syndrome may occur if the parietal lobes are compressed. decreased facial sensation. decreased hearing. deviation of the uvula. contralateral decreased pain sensation. to brain herniation. and involvement of bone and subcutaneous tissues by the meningioma. . compression of the underlying parenchyma. and hemiatrophy of the tongue. chordoid meningiomas can present with hematologic disturbances. and presence of the Babinski sign. o Compression of the nondominant (usually right) parietal lobe leads to tactile and visual extinction and neglect of the contralateral side. • Miscellaneous o Intraventricular meningiomas may present with obstructive hydrocephalus. right-left disorientation. o Meningiomas that compress the visual pathways produce various visual field defects. although rare.

the exact nature of this relationship and its implication on the management of meningiomas remain under investigation. 6q. the role of radiation in the genesis of meningiomas has been shown. posttraumatic insult) resulting in the upregulation of COX-2 has been investigated in the tumorogenesis of meningiomas. however. • Loss of chromosome 10 is associated with increased tumor grade. However. • The invasive potential of meningioma cells seems to be reflected by a balance between the expression of matrix metalloproteinases (MMPs) and tissue inhibitors of MMPs (TIMPs). 3p. the meningioma locus is close to but probably different from the gene responsible for NF2. • The most consistent chromosomal abnormality isolated in meningiomas is on the long arm of chromosome 22. the available data do not support such an association. • Several findings suggest an association between hormones and the risk for meningiomas. • Other cytogenetic alterations are chromosomal loss of 1p. • Of interest. progesterone. • High-dose cranial irradiation may induce meningiomas after a short latency period. especially with the recent tremendous increase in the use of these devices worldwide. At present. it is frequently reported in radiation-induced meningiomas. • Meningiomas can also be associated with different genetic syndromes. • Progression to anaplastic meningioma has been associated with involvement of chromosomal site 17q. • Whether cell phone use increases the risk of meningiomas (and of brain tumors in general) remains of great interest. and 14q. and shortened survival. all published studies have relatively small sample sizes and a short period of follow-up. However. increased expression of cyclooxygenase 2 and ornithine decarboxylase. • Patients subjected to low-dose irradiation for tinea capitis may develop multiple meningiomas decades later in the field of irradiation. and androgen receptors on some of these tumors. • The best-characterized and most common genetic alteration is the loss of the NF2 gene (NF2) on chromosome 22q[7] . • Genetic causes have been implicated in the development of meningiomas. namely Gorlin[8] and Rubinstein-Taybi syndromes[9] . • . including increased incidence in women versus men and the presence of estrogen. shortened time to recurrence. • Monosomy of chromosome 7 is a rare cytogenetic change.The role of inflammation (eg. loss of progesterone receptors. • Up to 60% of sporadic meningiomas were found to harbor NF2mutations. NF2 encodes a tumor suppressor known as merlin (or schwannomin). • The following events were found to be associated with higher grades of meningiomas: loss of the tumor suppressor in lung cancer-1 gene (TSLC-1).[6] • On the other hand.

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