Meningioangiomatosis with associated meningioma in a 4-year-old girl presenting with a focal seizure

Meningioangiomatosis is regarded as a rare, benign, hamartomatous malformation. Histopathologically, the lesion is characterized by circumscribed transcortical and leptomeningeal meningovascular proliferation with focal calcifications. It may be classified into cases with predominant cellular or vascular features and may occur in association with neurofibromatosis, mostly of type 2, but sporadic cases are more frequently reported. Sporadic cases often present initially with seizures and can be treated surgically. However, a certain percentage of patients will need ongoing anticonvulsive therapy. The lesions are seldom associated with an overlying meningioma. These are usually benign lesions that must be strictly separated from an invasive anaplastic meningioma, which would warrant an adjuvant therapy. We report on a 4-year-old girl who presented with spontaneous, predominantly cellular meningioangiornatosis with associated fibrous meningioma. Focal immunopositivity of the meningioangiomatosis for CD34 was helpful in ruling out an invasive meningioma.