TY - JOUR
T1 - Insights into meningioangiomatosis with without meningioma
T2 - A clinicopathologic end genetic series of 24 cases with review of the literature
AU - Perry, Arie
AU - Kurtkaya-Yapicier, Özlem
AU - Scheithauer, Bernd W.
AU - Robinson, Shenandoah
AU - Prayson, Richard A.
AU - Kleinschmidt-DeMasters, B. K.
AU - Stemmer-Rachamimov, Anat O.
AU - Gutmann, David H.
PY - 2005/1
Y1 - 2005/1
N2 - Meningioangiomatosis (MA) is a rare seizure-associated lesion of presumed hamartomatous or developmental origin. It is occasionally combined with a neoplasm, most commonly meningioma (MA-M). In the current study, we examined 24 cases (14 pure MA, 10 MA-M) using immunohistochemistry for merlin, protein 4.1 B, progesterone receptor (PR), and MIB-1, as well as FISH for NF2 and 4.1B gene dosages. Nine cases of MA-M (90%) had gene deletions (NF2/4.1B), protein losses (merlin/protein 4.1B), and/or PR positivity, with a similar or identical phenotype in both components. No PR positivity or gene deletions were seen in pure MAs, though merlin and/or protein 4.1B were immunonegative in six cases. Our data suggest that in most MA-Ms, the MA component is neoplastic, likely representing an exuberant perivascular pattern of spread from the meningioma, rather than an underlying hamartoma. This pattern of spread may be facilitated by meningiomas that are predominantly leptomeningeal or intracerebral in origin. It remains important to distinguish this pattern from true brain invasion, given the more ominous prognostic significance of the latter. In contrast, most perivascular spindled cells of pure MA are genetically and immunohistochemically similar to non-neoplastic meningothelial cells, consistent with current histogenetic theories.
AB - Meningioangiomatosis (MA) is a rare seizure-associated lesion of presumed hamartomatous or developmental origin. It is occasionally combined with a neoplasm, most commonly meningioma (MA-M). In the current study, we examined 24 cases (14 pure MA, 10 MA-M) using immunohistochemistry for merlin, protein 4.1 B, progesterone receptor (PR), and MIB-1, as well as FISH for NF2 and 4.1B gene dosages. Nine cases of MA-M (90%) had gene deletions (NF2/4.1B), protein losses (merlin/protein 4.1B), and/or PR positivity, with a similar or identical phenotype in both components. No PR positivity or gene deletions were seen in pure MAs, though merlin and/or protein 4.1B were immunonegative in six cases. Our data suggest that in most MA-Ms, the MA component is neoplastic, likely representing an exuberant perivascular pattern of spread from the meningioma, rather than an underlying hamartoma. This pattern of spread may be facilitated by meningiomas that are predominantly leptomeningeal or intracerebral in origin. It remains important to distinguish this pattern from true brain invasion, given the more ominous prognostic significance of the latter. In contrast, most perivascular spindled cells of pure MA are genetically and immunohistochemically similar to non-neoplastic meningothelial cells, consistent with current histogenetic theories.
UR - http://www.scopus.com/inward/record.url?scp=13544273530&partnerID=8YFLogxK
U2 - 10.1111/j.1750-3639.2005.tb00100.x
DO - 10.1111/j.1750-3639.2005.tb00100.x
M3 - Review article
C2 - 15779237
AN - SCOPUS:13544273530
SN - 1015-6305
VL - 15
SP - 55
EP - 65
JO - Brain Pathology
JF - Brain Pathology
IS - 1
ER -