MENINGIOANGIOMATOSIS: CLINICOPATHOLOGICAL EVALUATION OF A CASE

Öz

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.

Anahtar Kelimeler

• Meningioangiomatozis
• hamartoma
• nörofibromatozis

MENİNGİOANGİOMATOZİS: BİR OLGU ÜZERİNDEN KLİNİKOPATOLOJİK DEĞERLENDİRME

Öz

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).

Anahtar Kelimeler

• MENİNGİOANGİOMATOZİS
• BİR OLGU ÜZERİNDEN
• KLİNİKOPATOLOJİK
• DEĞERLENDİRME

Kaynakça

1. Burger PC, Scheithauer BW. Tumor-like lesions of maldevelopmental or uncertain origin. In Burger PC, Scheithauer BW (eds). Tumors of the Central Nervous System, AFIP. ARP press. Washington Dc, USA, 4nd ed., 2007; pp 493-95.
2. Wiebe S, Munoz DG, Smith S, Lee DH. Meningioangiomatosis: A comprehensive analysis of clinical and laboratory features. Brain. 1999; 122: 709Park MS, Suh DC, Choi WS, Lee SY, Kang GH. Multifocal meningioangiomatosis: A report of two cases. AJNR Am J Neuroradiol. 1999; 20: 677-80.
3. Omeis I, Hillard VH, Braun A, Benzil DL, Murali R, Harter DH. Meningioangiomatosis associated with neurofibromatosis: report of 2 cases in a single family and review of the literature. Surg Neurol. 2006; 65: 595-603.
4. Wang Y, Gao X, Yao ZW, Chen H, Zhu JJ, Wang SX, Gao MS, Zhou LF, Zhang FL. Histopathological study of five cases with sporadic meningioangiomatosis. Neuropathology. 2006; 26: 249Halper J, Scheithauer BW, Okazaki H, Laws ER Jr. Meningio-angiomatosis: a report of six cases with special reference to the occurrence of neurofibrillarytangles. J Neuropathol Exp Neurol. 1986; 45: 426-46.
5. Takeshima Y, Amatya VJ, Nakayori F, Nakano T, Sugiyama K, Inai K Meningioangiomatosis occurring in a young malewithout neurofibromatosis: with special reference to its histogenesis and loss of heterozygosity in the NF2 gene region. Am J Surg Pathol. 2002; 26: 125–129.
6. Perry A, Kurtkaya-Yapicier O, Scheithauer BW, Robinson S, Prayson RA, Kleinschmidt-DeMasters BK et al. Insights into meningioangiomatosis with and without meningioma: a clinicopathologic and genetic series of 24 cases with review of the literature. Brain Pathol. 2005; 15:55–65.
7. Kim NR, Cho SJ, Suh YL. Allelic loss on chromosomes 1p32, 9p21, 13q14, 16q22, 17p, and 22q12 in meningiomas associated with meningioangiomatosis and pure meningioangiomatosis. J Neurooncol. 2009; 94: 425