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2004, Number 5

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Rev Med Inst Mex Seguro Soc 2004; 42 (5)

Meningoangiomatosis: Case Report and Review of the Literature

Sánchez RG, Ángel RI, Muñoz TJM, Cruz MJ, Becerra LMM, Galindo ME

Language: Spanish
References: 17
Page: 413-418
PDF size: 782.94 Kb.


ABSTRACT

Meningoangiomatosis (MA) is a rare, benign, and possibly hamartomatous pathologic condition. Lesions may be single or diffuse and may be associated with type 2 neurofibromatosis (NF-2). Clinically, MA presents with seizures, but may also be asymptomatic or diagnosed at autopsy. Histologically, it is characterized by non-neoplastic proliferation of meningothelial cells involving subarachnoid space and adjacent cortex, with intralesional calcification. MA principally affects children and young adults < 20 years of age. We present the case of a neurologically sound 7-year-old boy with no pathologic history who presented with partial complex seizures with secondary generalization. Computerized tomography (CT) of head showed isodense lesion in left frontal lobe, with perilesional hypodensity and minimal contrast enhancement. In magnetic resonance imaging (MRI) of the brain, T1-weighted image showed isointense lesion with perilesional hypointensity, while T2-weighted image showed hyperintensity and homogeneous gadolinium enhancement. Left frontal craniotomy revealed thickened convolutions with a plane of separation between normal parenchyma and a moderately vascularized tumor of firm consistency, with cloudy meninges adhered to the affected cortex. The tumor was completely resected. Histologic analysis of the tumor revealed plaque-like proliferation of meningothelial cells in subarachnoid space that infiltrated cortex and underlying white matter. Meningothelial cells showed two different patterns of syncytial arrangement: a predominantly cellular pattern, and a predominantly vascular pattern, with collagen and reticulin deposition. At present, the patient is neurologically sound and seizure-free. The possible origin of the lesion is discussed and correlated with a review of the literature.


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