- Equal contributors
1 Department of Pathology, General Hospital of Jinan Military Command, Ji’nan 250031, China
2 Department of Pathology, State Key Laboratory of Cancer Biology, Xijing Hospital and School of Basic Medicine, Fourth Military Medical University, Xi’an 710032, China
3 Department of Radiology, Tangdu Hospital, Fourth Military Medical University, Xi’an 710038, China
BMC Neurology 2014, 14:32 doi:10.1186/1471-2377-14-32Published: 20 February 2014
Meningioangiomatosis (MA) is a rare hamartomatous lesion. Only six cases of cystic MA have been reported in the literature.
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.
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.