Meningioangiomatosis: The Pathological Spectrum of Lesions in Four Cases Including the First Report of a Fetal Case.
Sofia Hakim, Ellen Gelpi, Elena Martinez-Saez, Arantxa Ortega-Aznar, Alfons Nadal, Teresa Ribalta. Hospital Clinic, Barcelona, Spain; Hospital Vall d'Hebron, Barcelona, Spain
Background: Meningioangiomatosis (MA) is a rare, benign intracortical proliferation of meningothelial-like and fibroblast-like cells and vessels. It occurs in children and young adults with epilepsy. MA is more frequently sporadic than associated with NF2. MA is exceedingly rare in children under 3 years of age, and to date any case has been previously reported in a fetus. We have evaluated the clinicopathological characteristics of four cases, including the first reported case in a fetus, which illustrate the phenotypical diversity of this entity.
Design: We studied the clinical findings and surgical specimens of sporadic MA from three male patients (10, 16, 24 years) with a history of intractable seizures and the brain at autopsy of a 22-week-old male fetus. Clinical, neuroimaging and histopathological features, including immunostains for EMA, SMA, CD34, NeuN, Tau, betaA4, alpha-synuclein, TDP43, ubiquitin, and MIB-1, were evaluated.
Results: All the lesions were located in the frontal lobes and were heavily calcified except the fetal case. Microscopically, an extra-axial component made up of fascicles and whorls of fibroblastic-like cells with hyalinisation and calcification, and an intra-axial component with profuse vascular proliferation and perivascular meningothelial-like cells invading the brain were observed. In spite of the meningothelial-like morphology of perivascular cells, EMA staining was negative in all the cases. CD34 was positive in three lesions and SMA in two. The MIB1 LI was <0.1% in the four cases. Entrapped neurons demonstrated abundant tau+ (AT8+, 3R+, 4R+) neurofibrillary tangles and threads, especially in the three oldest patients.
Conclusions: MA is a rare, slow-growing meningocerebral lesion which neoplastic nature, histogenesis, and pathogenesis remain elusive. We report the first case in a fetus. Although morphologic features suggest a meningothelial differentiation, the inconstant immunostaining profile does not support this hypothesis. The lesion may be misinterpreted as an invasive meningioma, vascular malformation, astrocitoma or glioneuronal tumour, especially in the absence of adequate clinical information. The degenerative changes in the intervening cortex and calcification could be related to the age of the lesion.
Tuesday, March 1, 2011 1:00 PM
Poster Session IV # 237, Tuesday Afternoon