Meningioangiomatosis

Meningioangiomatosis is a rare, benign, hamartomatous lesion of the central nervous system (CNS). It is characterized by a proliferation of meningothelial cells, blood vessels, and neural elements. The condition is often associated with neurofibromatosis type 2 (NF2), but can also occur sporadically.

Clinical Presentation

Patients with meningioangiomatosis often present with seizures, headache, and focal neurological deficits. The age of onset varies, but the condition is most commonly diagnosed in children and young adults.

Pathology

Meningioangiomatosis is characterized by a proliferation of meningothelial cells, blood vessels, and neural elements. The lesion is often located in the cerebral cortex and leptomeninges, but can also occur in the spinal cord. Histologically, the lesion resembles a meningioma, but with a more complex architecture.

Diagnosis

The diagnosis of meningioangiomatosis is typically made based on histopathological examination of a biopsy specimen. Magnetic resonance imaging (MRI) can also be helpful in the diagnosis, as the lesion often shows a characteristic appearance on imaging.

Treatment

The treatment of meningioangiomatosis typically involves surgical resection of the lesion. In some cases, antiepileptic drugs may also be used to control seizures.

Prognosis

The prognosis for patients with meningioangiomatosis is generally good, as the lesion is benign and often responds well to treatment. However, the condition can be associated with significant morbidity due to seizures and other neurological deficits.

See Also

• Neurofibromatosis type 2
• Meningioma
• Central nervous system

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