Imaging and spectroscopic findings in meningioangiomatosis

Meningioangiomatosis (MA) is an uncommon brain tumor. The role of imaging techniques is underscored in cases where the tumor location makes resection (or even biopsy) dangerous. We report the case of a child with an MA tumor located deep in the right sylvian fissure. A computed tomography (CT) scan showed calcifications in a highly vascular lesion with surrounding edema. Magnetic resonance spectroscopy (MRS) showed a distinct choline (Cho) peak, which usually suggests a proliferating tumor. Fluorodeoxyglucose positron emission tomography (FDG‐PET) showed the lesion lacked hypermetabolic features. These radiological features should put MA in the differential diagnosis. Pediatr Blood Cancer 2009;53:672–674. © 2009 Wiley‐Liss, Inc.     

Histopathological study of five cases with sporadic meningioangiomatosis

We report five cases of sporadic meningioangiomatosis, three males and two females, ranging in age from 12 to 36 years at diagnosis. The lesion was found incidentally by MRI after a head trauma in one case; the other four subjects had a seizure disorders, which improved following surgical resection of the cortical lesions. Grossly, the lesionectomy specimens were of a whitish color and firm consistency. Histological examination revealed that the lesions were confined to the cortex with focal involvement of the overlying leptomeninges, and revealed unifying features of meningioangiomatosis, such as proliferating microvessels with perivascular cuffs of spindle‐cell proliferation within the cortex. Two cases had numerous calcifications; one was associated with a prominent fibrocalcifying component. Immunostaining results were variable among the cases. Only vimentin was consistently positive. Some of the spindle cells were weak positive for EMA in two cases. Immunoreactions with anti‐CD34 detected within the cytoplasm of the spindle cells were observed in three of the five cases. The Ki‐67 proliferation index of all the cases was very low, less than 0.1%. Neurofibrillary tangles were identified in only one of the five cases using the Bodian and immunostaining methods. These findings indicate that meningioangiomatosis lesions show a wide range of clinicopathological features, making diagnosis difficult. A histopathological spectrum and differential diagnoses were discussed with a review of the literature. Since this lesion is a distinct clinicopathological entity and hamartomatous in nature, it is important to make a correct diagnosis in order to avoid further aggressive treatment.     

颅内脑膜血管瘤一例并文献复习

目的探讨脑膜血管瘤病（MA）的临床病理特征。方法分析1例MA病史及影像学、病理学资料并复习相关文献。结果MA病理学特点为细胞区脑膜细胞呈结节状、漩涡状或带状增生；血管区以增生小血管及其周围的梭形纤维母细胞样细胞为典型特点。免疫组化结果显示，vimentin及上皮膜抗原（EMA）阳性，胶质纤维酸性蛋白（GFAP）及S-100蛋白阴性。电镜检查结果显示，肿瘤细胞有细胞间桥粒和胞突犬牙交错及胞质内众多微丝等脑膜上皮的特征。结论MA是一种罕见疾病，分为散发型和2型神经纤维瘤病相关型（NF2）。MA病变通常位于大脑额颞叶皮质层并可累及周围脑膜组织。MA在形态学上应与侵袭性脑膜瘤、节细胞神经瘤等相鉴别，病变组织生长方式、免疫组化表型和电镜特点在该肿瘤的诊断和鉴别诊断中具重要作用。     

脑膜血管瘤病的临床特点（附1例报道）

目的探讨脑膜血管瘤病的临床特点方法对我院收治的1例脑膜血管瘤病的临床表现、影像和病理特点进行了回顾性分析。结果脑膜血管瘤病常在青少年起病,以癫癎多见,病理上发现病灶多位于大脑皮层内,累及软脑膜,伴有脑膜血管增生。手术切除后癫癎控制满意。结论脑膜血管瘤病是颅内较为罕见的良性病变,手术切除是治疗本病的主要手段。     

脑膜血管瘤病合并脑膜瘤临床病理及文献复习

目的 探讨脑膜血管瘤病(MA)合并脑膜瘤的临床病理特点.方法 回顾性分析1例颅内MA合并脑膜瘤病人的临床资料,行病理形态学观察及免疫组织化学检测,并对该病进行文献复习.结果 光镜观察:皮质内血管增生,脑膜上皮细胞及梭形的纤维母细胞样细胞围绕血管排列,部分大血管明显胶原化及透明变性,伴钙化或砂砾体形成,且可见明显的过渡型脑膜瘤成分,病灶内残留变性神经元和胶质细胞增生.免疫组化染色显示:vimentin阳性,上皮细胞膜抗原(EMA)局灶性阳性,Ki-67阳性率在脑膜瘤区域约为3％,在脑膜血管瘤病区则＜1％.结论 MA是--种少见的良性病变,病变通常位于大脑额颞叶皮质并可累及周围脑膜组织,MA合并脑膜瘤更少见,在形态学上应与侵袭性脑膜瘤、孤立性纤维瘤、颅内神经鞘膜瘤等鉴别.     

脑膜血管瘤病临床病理特点及免疫组化分析

目的提高对脑膜血管瘤病（meningioangiomatosis,MA）的临床病理特征的认识,减少漏诊和误诊。方法复习4例MA的临床表现、影像学、病理形态学及免疫纽化特征和相关文献。结果4例均为12岁以前发病,以难治性癫痫发作为主要症状。影像学均有胶质增生、钙化、轻度占位效应及瘤组织周围轻度水肿。组织病理学表现为以脑膜上皮细胞和纤维母细胞样梭形细胞增生并围绕脑膜和皮层内的小血管周围,伴有钙化或砂粒体形成,病灶内残留变性的神经元和胶质细胞增生。免疫组化染色vimentin阳性,EMA灶性阳性,GFAP、NF、Syn均阴性。结论MA是以软脑膜钙化及脑膜血管增生为特征的良性大脑皮层病变。影像学表现为瘤周水肿和胶质增生。免疫组化对诊断MA作用不大。     

脑膜血管瘤病临床病理观察

目的：认识脑膜血管瘤病的临床病理表现和本质。方法：复习3例患者的临床病史，影像学表现及病理形态，免疫组化染色（ABC法）抗体包括：EMA,vimentin、SMA、GFAP、Syn及相关文献。结果：3例均为男性，2例为少年，均有癫痫发作，另1例为偶有头痛，均经CT或MRI扫描而发现病变，病变位于颞后近颞顶枕三角区的皮层内，皮层内充满围绕小血管生长的纤维母细胞样梭形细胞或脑膜上皮细胞，免疫组化染色EMA、vimentin阳性，有不同程度和数量的砂粒小体或钙化形成，病灶内残留神经细胞和胶质增生。结论：从病变的临床表现看，本组3例可能均属于散发型脑膜血管瘤病，而非2型神经纤维瘤病（NF2）相关病例。     

Sudden, Unexpected Death Associated with Meningioangiomatosis: Case Report

We report a case of sudden, unexpected death associated with meningioangiomatosis in a 13-year-old, previously healthy male without a history of seizures, neurologic deficits, or clinical stigmata of neurofibromatosis. There was no family history of neurofibromatosis. The postmortem examination showed a 5-cm mass involving the right posterior frontal and orbital frontal cortex that had microscopic features diagnostic of meningioangiomatosis. Because no other cause of death was found, we postulate that he likely died as a result of a seizure secondary to meningioangiomatosis.     

ILAE focal cortical dysplasia type IIIc in the ictal onset zone in epileptic patients with solitary meningioangiomatosis

“Solitary” meningioangiomatosis (MA) is a rare, benign, hamartomatous lesion of the cerebral cortex and frequently leads to epilepsy. However, the source of the epileptogenicity in meningioangiomatosis remains controversial. We report two surgically‐treated meningioangiomatosis cases with medically intractable epilepsy. In both cases, chronic subdural electrocorticogram (ECoG) recordings identified the ictal onset zone on apparently normal cortex, adjacent to and/or above the meningioangiomatosis lesion, not on the meningioangiomatosis lesion itself. The ictal onset zone was resected, along with the MA lesion, and good seizure outcome was achieved. Histological examination of the ictal onset zone revealed the presence of ILAE focal cortical dysplasia (FCD) type IIIc. Our case studies suggest that in the surgical management of epilepsy with meningioangiomatosis, it is important to identify undetected, but epileptogenic, ILAE FCD Type IIIc, using preoperative multimodal examinations, including chronic ECoG recordings.