Meningioangiomatosis

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Meningioangiomatosis

Meningioangiomatosis (pronunciation: meh-nin-gee-oh-an-gee-oh-ma-toe-sis) is a rare, benign lesion of the central nervous system that primarily affects the cerebral cortex. It is often associated with neurofibromatosis type 2 (NF2), but can also occur sporadically.

Etymology

The term "meningioangiomatosis" is derived from the Greek words "meninx" (meaning membrane), "angeion" (meaning vessel), and "oma" (meaning tumor). The suffix "-osis" indicates a condition or process. Thus, the term refers to a condition involving abnormal proliferation of the meningeal and vascular tissues.

Clinical Presentation

Patients with meningioangiomatosis often present with seizures, headaches, and focal neurological deficits. The condition is typically diagnosed through magnetic resonance imaging (MRI) and confirmed with histopathological examination.

Pathology

Meningioangiomatosis is characterized by a proliferation of meningothelial cells, leptomeningeal cells, and blood vessels within the cerebral cortex. The lesion often infiltrates the surrounding brain tissue, leading to cortical atrophy and calcification.

Treatment

Treatment for meningioangiomatosis primarily involves surgical resection of the lesion. In cases associated with NF2, additional treatment may be required for associated tumors such as vestibular schwannomas and meningiomas.

Prognosis

The prognosis for patients with meningioangiomatosis is generally good, particularly for those with sporadic cases. However, the prognosis can be more variable for patients with NF2-associated meningioangiomatosis, depending on the presence and severity of other associated tumors.

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