title: Multicystic meningioangiomatosis.
abstract: BACKGROUND
  Meningioangiomatosis (MA) is a rare hamartomatous lesion. Only six cases of cystic MA have been reported in the literature.
  
  
  METHODS
  We present a case of multicystic MA. A 21-year-old woman without any stigmata of neurofibromatosis type 2 presented with intractable seizures since 10 years. Brain magnetic resonance imaging revealed a well-defined, multicystic mass with heterogeneous signal intensity in the right temporal lobe. The patient underwent resection of the lesion and of the epileptogenic cortex under intraoperative electrocorticography (ECoG) assistance. Histopathological examination showed proliferation of perivascular cells that were arranged in a cuff pattern and were positive for vimentin, D2-40 and smooth muscle actin. Mutiple microcysts and enlarged perivascular spaces were present, which was similar to the structure of the arachnoid cavity. Hyalinized collagen fibers with round concentric acellular eosinophilic lamellae within areas of reactive gliosis were noted for the first time in MA. The patient was followed up without any clinical symptoms or recurrence for 2 years.
  
  
  CONCLUSIONS
  MA may originate from arachnoid and vascular tissue trapped in the cortical parenchyma during brain development, and the cysts may have resulted from the gradual accumulation of cerebrospinal fluid in the perivascular spaces of the trapped tissue. Resection of the lesion and of the epileptogenic cortex is important not only for pathological diagnosis but also for seizure control, and intraoperative ECoG assistance is recommended.
authors.forename: Peifeng
authors.initials: P
authors.lastname: Li
authors.forename: Guangbin
authors.initials: G
authors.lastname: Cui
authors.forename: Yingmei
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Department of Pathology, General Hospital of Jinan Military Command, Ji'nan 250031, China. gm2227@sina.com.
authors.forename: Ming
authors.initials: M
authors.lastname: Geng
authors.forename: Zhe
authors.initials: Z
authors.lastname: Wang
mesh_terms: D020863:Central Nervous System Cysts / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D005260:Female; D006801:Humans; D008577:Meningeal Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D008579:Meningioma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D055815:Young Adult