Diagnosis and surgical treatment of sporadic meningioangiomatosis

Feng, Rui; Hu, Jie; Che, Xiaoming; Pan, Li; Wang, Zhiqiu; Zhang, Mingguang; Huang, Fei; Xu, Bin; Mao, Ruolin; An, Sun; Bao, Weimin; Zhong, Ping; Wang, Yin

doi:10.1016/j.clineuro.2013.01.021

Cited by 14 publications

(8 citation statements)

References 19 publications

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“…Extra-axial tumors, such as meningiomas, are the most common differential diagnosis. Intra-axial tumors in the superficial cortex, such as oligodendroglioma, ganglioglioma, and dysembryoplastic neuroepithelial tumor (DNET), are also frequently considered [ 24 ]. However, for cystic MA, we thought that differential tumors should include some cystic-mural tumors with non-enhanced cystic walls, such as hemangioblastomas, pilocystic astrocytomas, and pleomorphic xanthoastrocytomas.…”

Section: Case Presentationmentioning

confidence: 99%

Three cases of sporadic meningioangiomatosis with different imaging appearances: case report and review of the literature

et al. 2015

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BackgroundMeningioangiomatosis (MA) is a rare meningiovascular malformation or hamartomatous lesion in the central nervous system. Radiographic findings of MA may show a variety of characteristics according to different histological components. We present three cases of sporadic MA with different imaging appearances in an attempt to identify specific imaging characteristics.Case presentationIn case 1, an irregular hyperdense solid mass was localized in the left middle cranial fossa, demonstrating low and equal signal intensity on T1-weighted imaging (T1WI; TR/TE 2,048.9 ms/26.1 ms), high signal intensity with multiple flow void effect on T2-weighted imaging (T2WI; TR/TE 4,000 ms/106.4 ms), and significant and homogeneous enhancement on post-contrast magnetic resonance imaging (MRI). In case 2, the lesion in the right insular lobe showed a cystic-mural nodule pattern. The cystic content demonstrated similar density or signal intensity as cerebrospinal fluid, while the mural nodule demonstrated equal density or signal intensity on computed tomography (CT) and MRI. On post-contrast MRI, the mural nodule showed significant enhancement, but the cystic wall and content showed no enhancement. In case 3, a remarkably enhanced solid nodule was found in the cortex of the left parietal lobe with multiple small cysts surrounding it. This nodule showed low signal intensity on T2WI and diffusion-weighted imaging (DWI; TR/TE 6,000 ms/96.8 ms, b = 1,000 s/mm2). The preoperative diagnoses of the above three cases were meningioma, hemangioblastoma, and ganglioglioma. However, all were pathologically diagnosed as MA.ConclusionThe presented cases demonstrate that MA may present with solid and cystic imaging patterns, which may include large cystic-mural nodules and small intra- and extra-cystic patterns. Although MA imaging diagnoses are difficult, several MRI signs may include specific characteristics, such as a flow void effect on T2WI and separating cysts in the cystic MA (as shown in our cases), gyriform hyperintensity on T2-fluid attenuated inversion recovery (FLAIR) sequence, and susceptibility artifacts on T2 gradient echo (GRE) sequences (as found in the literature).

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Section: Case Presentationmentioning

confidence: 99%

Three cases of sporadic meningioangiomatosis with different imaging appearances: case report and review of the literature

et al. 2015

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“…Information regarding MRS of MA is very rare. In the literature, MRS revealed slightly higher Cho/NAA ratio compared to normal brain parenchyma [1,6,8]. Reduced Cho/Cr and Cho/NAA ratios in this case may be attributed to the hyalinization and low proliferative activity, which was also supported by low Ki-67 labeling index.…”

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confidence: 67%

Meningioangiomatosis of the cerebellum: radiopathologic characteristics of a case

et al. 2015

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Dear Editor, Meningioangiomatosis (MA) is a rare hamartomatous lesion involving cortex and overlying leptomeninges. It is characterized by cortical vascular proliferation with perivascular cuffs of proliferative spindle-shaped cells [9]. Most cases are sporadic, but association with NF type 2 is well known [2,5]. MA mainly involves the cerebral cortex, whereas unusual locations such as thalamus and the brainstem have also been reported [3,4,10]. Omeis et al. reported a cerebellar MA in a patient with NF type 2, but the lesion was not radiologically demonstrated [7]. Here we present not only the first sporadic case of cerebellar MA but also the first radiologically documented one.A 55-year-old woman was admitted to the neurosurgery outpatient clinic with the symptom of vertigo for 2 months. She had no other known medical conditions and no family history of NF 2. Her physical and neurological examinations were unremarkable. MRI revealed a cortical inhomogeneous lesion with well-defined but irregular borders in left superior cerebellum. Peripheral rim of T1 hyper, T2 hypo-intensity, and susceptibility on T2* GE images were suggestive of calcification (Fig. 1a, c). MRI also detected small satellite cortical and subcortical cysts (Fig. 1a, b). The lesion showed peripheral irregular enhancement with no perilesional edema (Fig. 1b). Elevated diffusion with a high apparent diffusion coefficient (ADC) value (Fig. 1d), low myo-inositol in single voxel (TE: 30 ms) proton MR spectroscopy (MRS) suggested low cellularity and low astrocytic component, respectively. Multi-voxel (TE: 270 ms) MRS revealed moderately reduced N-acetyl-aspartate (NAA), NAA/creatine (Cr) ratio (1.06), choline, Cho/Cr ratio (0.56), Cho/NAA ratio (0.52). Lactate peak was also present. Perfusion MRI showed low relative cerebral blood volume (rCBV: 0.84), low relative cerebral blood flow (rCBF: 0.34), and increased relative mean transit time (rMTT: 2.3) (Fig. 1e). The patient underwent surgery with lateral supracerebellar approach. A neuronavigation system was used to detect the exact location. After a left retrosigmoid craniotomy, the lesion was explored on the superior cerebellar surface. The lesion was firm, similar to a meningioma; however it was totally intraparenchymal with no dural connection. Gross total resection using microsurgical techniques was performed. The postoperative course was uneventful with no operation-related morbidity. Histologically, the cerebellar cortex was variably replaced by fibrous tissue and hyalinized small-sized vessels coated with subtle proliferations of spindle cells (Fig. 1f). There were excessive psammoma bodies within the lesion and the surrounding parenchyma. Immunohistochemical examination revealed perivascular weak expressions of EMA (a meningothelial cell marker), vimentin (a non-specific mesenchymal cell marker) and Ki-67 labeling index of <1 %.MA can be broadly classified into cellular and vascular patterns according to the predominant component [10]. Over time, fibrocalcifying changes develop, and even osseou...

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“…The lesions are often associated with NF2, although the majority of cases are sporadic [8]. Sporadic MA usually occurs as a solitary lesion in young patients and presents with a history of intractable seizures or persistent headaches [9,10]. In contrast, NF2-associated MA more frequently involves multiple lesions and is asymptomatic [11].…”

Section: Discussionmentioning

confidence: 99%

Multicystic meningioangiomatosis

Cui

Wang

et al. 2014

BMC Neurol

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BackgroundMeningioangiomatosis (MA) is a rare hamartomatous lesion. Only six cases of cystic MA have been reported in the literature.Case presentationWe 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.ConclusionMA 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.

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Diagnosis and surgical treatment of sporadic meningioangiomatosis

Cited by 14 publications

References 19 publications

Three cases of sporadic meningioangiomatosis with different imaging appearances: case report and review of the literature

Three cases of sporadic meningioangiomatosis with different imaging appearances: case report and review of the literature

Meningioangiomatosis of the cerebellum: radiopathologic characteristics of a case

Multicystic meningioangiomatosis

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