MENINGIOANGIOMATOSIS: CLINICOPATHOLOGICAL EVALUATION OF A CASE

Abstract

Meningioangiomatosis (MA) is a rare, benign, focal lesion of leptomeninges and the underlying cerebral cortex, characterized by perivascular meningothelial and fibroblastic cell proliferation. It may be seen either sporadically or in patients with Neurofibromatosis-2. MA is considered to be a hamartomatous or maldevelopmental lesion, a reactive condition, or a lesion of neoplastic origin. This lesion is important since the differential diagnosis should be made for intracortical tumors. In this case report we present an 18 year-old male patient who had seizures for five months. He has not developed any clinical symptoms after surgical excision of the lesion. This rare case is discussed clinically and morphologically with literature findings.

Keywords

• Meningioangiomatozis
• hamartoma
• nörofibromatozis

MENİNGİOANGİOMATOZİS: BİR OLGU ÜZERİNDEN KLİNİKOPATOLOJİK DEĞERLENDİRME

Abstract

Meningioangiomatozis (MA), intrakortikal meningotelyal ve fibroblastik hücrelerin perivasküler proliferasyonudur (1). İlk olarak 1915 yılında Bassoe ve Nuzum tarafından von Recklinghausen hastalığıyla ilişkili olarak bildirilmiştir. Ayrıca sporadik olarak da görülürler. Klinik olarak hastalar sıklıkla parsiyel nöbetle gelirler (2). MA patogenezi bilinmemekle birlikte bazı teoriler öne sürülmüştür. Bunlardan biri Virchow-Robin boşluklarındaki fibroblastik ve araknoidal hücrelerin proliferasyonuyla serebral kortekste bir mikrovasküler malformasyonun sonucu oluştuğudur. Diğer bir teori leptomeningial orjinli meningiomun direkt beyin parankimine invazyonu sonucu ortaya çıkan morfolojik görünüm olabileceğidir. Ayrıca dejeneratif değişikler sonucu ortaya çıkan meningovasküler bir hamartom olduğu görüşü de ileri sürülmüştür (2,3). MA’lar reaktif veya hamartomatöz gelişimler olarak düşünülmekle birlikte bazen meningiomla birlikteliği ve bu olgularda saptanan moleküler değişiklikler bunların tümöral olabileceğine işaret etmektedir (1).

Keywords

• MENİNGİOANGİOMATOZİS
• BİR OLGU ÜZERİNDEN
• KLİNİKOPATOLOJİK
• DEĞERLENDİRME

References

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