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INTRACRANIAL NEOPLASMS AND PARANEOPLASTIC DISORDERS
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sites of predilection for the tumor Grossly, the tumor is rm, gray, and sharply circumscribed, taking the shape of the space in which it grows; thus, some tumors are at and plaque-like, others round and lobulated They may indent the brain and acquire a pia-arachnoid covering as part of their capsule, but they are clearly demarcated from the brain tissue (extra-axial) except in the unusual circumstance of a malignant invasive meningioma Rarely, they arise from arachnoidal cells within the choroid plexus, forming an intraventricular meningioma Microscopically, the cells are relatively uniform, with round or elongated nuclei, visible cytoplasmic membrane, and a characteristic tendency to encircle one another, forming whorls and psammoma bodies (laminated calci c concretions) A notable electron microscopic characteristic is the formation of very complex interdigitations between cells and the presence of desmosomes (Kepes) Cushing and Eisenhardt and, more recently, the World Health Organization (Lopes et al) have divided meningiomas into many subtypes depending on their mesenchymal variations, the character of the stroma, and their relative vascularity, but the value of such classi cations is debatable Currently neuropathologists recognize a meningothelial (syncytial) form as being the most common It is readily distinguished from other similar but non-meningothelial tumors such as hemangiopericytomas, broblastomas, and chondrosarcomas The usual sites of meningioma are the sylvian region, superior parasagittal surface of the frontal and parietal lobes, olfactory groove, lesser wing of the sphenoid bone, tuberculum sellae, superior surface of the cerebellum, cerebellopontine angle, and spinal canal Some meningiomas such as those of the olfactory groove, sphenoid wing, and tuberculum sellae express themselves by highly distinctive syndromes that are diagnostic in themselves; these are described further on in this chapter Rarely, they are mul-
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tiple Inasmuch as they extend from the dural surface, they often invade and erode the cranial bones or excite an osteoblastic reaction, even giving rise to an exostosis on the external surface of the skull The following remarks apply to meningiomas of the parasagittal, sylvian, and other surface areas of the cerebrum Small meningiomas, less than 20 cm in diameter, are often found at autopsy in middle-aged and elderly persons without having caused symptoms Only when they exceed a certain size and indent the brain do they alter function The size that must be reached before symptoms appear varies with the size of the space in which the tumor grows and the surrounding anatomic arrangements Focal seizures are often an early sign of meningiomas that overlie the cerebrum The parasagittal frontoparietal meningioma may cause a slowly progressive spastic weakness or numbness of one leg and later of both legs, and incontinence in the late stages The sylvian tumors are manifest by a variety of motor, sensory, and aphasic disturbances in accord with their location, and by seizures In the past, before brain imaging techniques became available, the meningioma often gave rise to neurologic signs for many years before the diagnosis was established, attesting to its slow rate of growth Even now some tumors reach enormous size, to the point of causing papilledema, before the patient comes to medical attention A few may be detected in plain lms or in CT scans in individuals with unrelated neurologic diseases Increased intracranial pressure eventually occurs, but it is less frequent with meningiomas than with gliomas As mentioned above, the diagnosis of meningiomas is greatly facilitated by their ready visualization with contrast-enhanced CT and MRI (Figs 31-7 and 31-8) and by arteriography, all of which reveal their tendency to calcify and their prominent vascularity These changes are re ected by homogeneous contrast enhancement
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Figure 31-7 A Falx meningioma; coronal image, MRI with gadolinium B Sphenoid wing meningioma Contrast-enhanced CT scan
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