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Vol. 17. Issue 3.
Pages 250-254 (January 2006)
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Vol. 17. Issue 3.
Pages 250-254 (January 2006)
Meningioangiomatosis. Descripción de dos casos y revisión de la literatura
Meningioangiomatosis: report of two cases and literature review
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A. Suárez-Gauthier, M.R. Gómez de la Bárcena, E. García-García, J.R. Ricoy
Departamentos de Anatomía Patológica/Neuropatología. Hospital Universitario 12 de Octubre. Madrid
J. Hinojosa*
*. Departamentos de Neurocirugía infantil. Hospital Universitario 12 de Octubre. Madrid
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Resumen

La Meningioangiomatosis (MA) es una lesión benigna intracraneal poco frecuente. La mayoría de los casos son aislados, pero también se han descrito casos asociados a Neurofibromatosis (NF) tipo 2. Los casos asociados a NF suelen ser asintomáticos. Los casos no asociados a NF son sintomáticos, se presentan en niños y adultos jóvenes, y en general afectan a leptomeninge y a corteza cerebral subyacente.

En este trabajo se describen 2 casos no asociados a NF en niños de 1 y 7 años de edad, que se manifestaron por crisis, las cuales desaparecieron tras la extirpación de la lesión. Histológicamente las lesiones eran de predominio celular en un caso y de predominio fibro-hialino en el otro.

De la revisión de la literatura se concluye que las meningioangiomatosis no asociadas a NF suelen ser lesiones únicas, clínicamente suelen presentarse con crisis convulsivas, y pueden verse asociadas a otras patologías, fundamentalmente a meningiomas.

Histológicamente las lesiones corresponden a una proliferación celular de hábito meningotelial que se dispone alrededor de los vasos corticales. A pesar de que todos los casos tienen características comunes, existe un amplio espectro de formas histológicas, que abarca desde casos más celulares hasta casos más fibrosos con calcificaciones. Estas diferencias podrían corresponder a diferentes estadios evolutivos de la lesión. La extirpación de la lesión conlleva la desaparición de los síntomas.

Palabras clave:
Corteza cerebral
Hamartoma, Leptomeninge
Meningioangiomatosis
Meningioma
Tumor
Abreviaturas:
MA
NF
Summary

Meningioangiomatosis (MA) is a rare benign intracraneal lesion. The majority of cases are sporadic although the association of this lesion with familial neurofibromatosis (NF) type 2 is well known. NF-associated MA may be multifocal and is often asymptomatic and diagnosed at autopsy. Non-associated cases are usually symptomatic, occurs in children and young adults and frequently arise in leptomeninges and underlying cerebral cortex.

In the present work, we describe two new non-associated cases of MA in two boys, seven and one year old with seizures that disappeared after surgical excision.

Histopathologically, the lesion was predominantly cellular in one case and more fibrous in the other.

From the literature review we concluded that sporadic cases present as single lesions which manifest by seizures or persistent headaches. Rarely MA has been described to coexist with meningiomas.

Histopathologically, MA is characterized by a plaque-like proliferation of meningothelial and fibroblast- like cells surrounding small vessels and trapping islands of gliotic cortical tissue. The lesion does not show significant atypia, mitosis or necrosis. Although all cases of MA share unifying features, there are different degrees of histological presentation with cases predominantly cellular and others more fibrous and calcified. This could correspond to different stages in the evolution of the MA. Symptoms disappear with the complete excision of the lesion.

Key words:
Cerebral cortex
Hamartoma
Leptomeninge
Meningioangiomatosis
Meningioma
Tumour

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