Insights into meningioangiomatosis with without meningioma: A clinicopathologic end genetic series of 24 cases with review of the literature

Arie Perry, Özlem Kurtkaya-Yapicier, Bernd W. Scheithauer, Shenandoah Robinson, Richard A. Prayson, B. K. Kleinschmidt-DeMasters, Anat O. Stemmer-Rachamimov, David H. Gutmann

Research output: Contribution to journalReview article

Abstract

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.

Original languageEnglish (US)
Pages (from-to)55-65
Number of pages11
JournalBrain Pathology
Volume15
Issue number1
StatePublished - Jan 2005
Externally publishedYes

Fingerprint

Neurofibromin 2
Meningioma
Progesterone Receptors
Gene Deletion
Proteins
Hamartoma
Gene Dosage
Seizures
Immunohistochemistry
Phenotype
Brain
Neoplasms

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine

Cite this

Perry, A., Kurtkaya-Yapicier, Ö., Scheithauer, B. W., Robinson, S., Prayson, R. A., Kleinschmidt-DeMasters, B. K., ... Gutmann, D. H. (2005). Insights into meningioangiomatosis with without meningioma: A clinicopathologic end genetic series of 24 cases with review of the literature. Brain Pathology, 15(1), 55-65.

Insights into meningioangiomatosis with without meningioma : A clinicopathologic end genetic series of 24 cases with review of the literature. / Perry, Arie; Kurtkaya-Yapicier, Özlem; Scheithauer, Bernd W.; Robinson, Shenandoah; Prayson, Richard A.; Kleinschmidt-DeMasters, B. K.; Stemmer-Rachamimov, Anat O.; Gutmann, David H.

In: Brain Pathology, Vol. 15, No. 1, 01.2005, p. 55-65.

Research output: Contribution to journalReview article

Perry, A, Kurtkaya-Yapicier, Ö, Scheithauer, BW, Robinson, S, Prayson, RA, Kleinschmidt-DeMasters, BK, Stemmer-Rachamimov, AO & Gutmann, DH 2005, 'Insights into meningioangiomatosis with without meningioma: A clinicopathologic end genetic series of 24 cases with review of the literature', Brain Pathology, vol. 15, no. 1, pp. 55-65.
Perry A, Kurtkaya-Yapicier Ö, Scheithauer BW, Robinson S, Prayson RA, Kleinschmidt-DeMasters BK et al. Insights into meningioangiomatosis with without meningioma: A clinicopathologic end genetic series of 24 cases with review of the literature. Brain Pathology. 2005 Jan;15(1):55-65.
Perry, Arie ; Kurtkaya-Yapicier, Özlem ; Scheithauer, Bernd W. ; Robinson, Shenandoah ; Prayson, Richard A. ; Kleinschmidt-DeMasters, B. K. ; Stemmer-Rachamimov, Anat O. ; Gutmann, David H. / Insights into meningioangiomatosis with without meningioma : A clinicopathologic end genetic series of 24 cases with review of the literature. In: Brain Pathology. 2005 ; Vol. 15, No. 1. pp. 55-65.
@article{455ccc1f1c484d64b7fc17e360621d8c,
title = "Insights into meningioangiomatosis with without meningioma: A clinicopathologic end genetic series of 24 cases with review of the literature",
abstract = "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.",
author = "Arie Perry and {\"O}zlem Kurtkaya-Yapicier and Scheithauer, {Bernd W.} and Shenandoah Robinson and Prayson, {Richard A.} and Kleinschmidt-DeMasters, {B. K.} and Stemmer-Rachamimov, {Anat O.} and Gutmann, {David H.}",
year = "2005",
month = "1",
language = "English (US)",
volume = "15",
pages = "55--65",
journal = "Brain Pathology",
issn = "1015-6305",
publisher = "Wiley-Blackwell",
number = "1",

}

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

UR - http://www.scopus.com/inward/citedby.url?scp=13544273530&partnerID=8YFLogxK

M3 - Review article

C2 - 15779237

AN - SCOPUS:13544273530

VL - 15

SP - 55

EP - 65

JO - Brain Pathology

JF - Brain Pathology

SN - 1015-6305

IS - 1

ER -