Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases (NCRC-ND), Center of Brain Tumor, Beijing Key Laboratory of Brian Tumor, Fengtai District, Beijing Institute for Brain Disorders, Beijing Tiantan Hospital, Capital Medical University, South 4th Ring West Road 119, 100050, Beijing, People's Republic of China.
Department of Critical Care Medicine, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, People's Republic of China.
Neurosurg Rev. 2022 Oct;45(5):3405-3415. doi: 10.1007/s10143-022-01855-8. Epub 2022 Sep 5.
Meningioangiomatosis (MA) is a disease that is extremely rarely reported. Sporadic MA is occasionally combined with meningioma or other lesions (identified as non-pure MA). This retrospective study investigated the difference between pure MA and non-pure MA by exploring clinical manifestations, histopathology characteristics, and outcomes of MA after surgery. We reviewed the medical records of 36 histopathologically confirmed MA patients (18 pure MA and 18 non-pure MA) who received surgery at our institution between 2012 and 2021. We compared differences in demographic, clinical, imaging, pathological features, and surgical outcomes between pure MA and non-pure MA through descriptive statistics. Compared to non-pure MA, pure MA presented with a more prominent male predilection (5:1 vs. 1.57:1, P = 0.264), a higher seizure incidence (83.3% vs 50.0%, P = 0.038), a more seizure type of GTCS (14/15 vs 5/9, P = 0.047), a less prominent enhancement on MRI (27.8% vs 88.9%, P < 0.001) and a preference of temporal and frontal lobe (100% vs 44.4%, P < 0.001). The differences in clinical characteristics between pure MA and non-pure MA demonstrate their disparate biological natures. Pure MA seems to be a non-neoplastic lesion, while non-pure MA is commonly combined with meningioma, which is a neoplastic lesion. A correct differential diagnosis can be achieved via a triad of the type of seizure, the location of lesion and the radiological presentation. MA is curable and the prognosis is excellent as most patients are free of seizure and recurrence after surgical treatment.
脑膜血管外皮细胞瘤病（MA）是一种极为罕见的疾病。散发性 MA 偶尔与脑膜瘤或其他病变（鉴定为非纯 MA）合并。本回顾性研究通过探索 MA 手术后的临床表现、组织病理学特征和结局，来研究纯 MA 和非纯 MA 之间的差异。我们回顾了 2012 年至 2021 年在我们机构接受手术的 36 例组织学确诊的 MA 患者（18 例纯 MA 和 18 例非纯 MA）的病历。我们通过描述性统计比较了纯 MA 和非纯 MA 之间的人口统计学、临床、影像学、病理学特征和手术结局的差异。与非纯 MA 相比，纯 MA 表现出更明显的男性偏好（5:1 比 1.57:1，P=0.264）、更高的癫痫发生率（83.3%比 50.0%，P=0.038）、更常见的 GTCS 癫痫类型（14/15 比 5/9，P=0.047）、MRI 增强程度较轻（27.8%比 88.9%，P<0.001）和更倾向于颞叶和额叶（100%比 44.4%，P<0.001）。纯 MA 和非纯 MA 之间的临床特征差异表明它们具有不同的生物学性质。纯 MA 似乎是非肿瘤性病变，而非纯 MA 通常与脑膜瘤合并，脑膜瘤是一种肿瘤性病变。通过癫痫发作类型、病变部位和影像学表现的三联征可以做出正确的鉴别诊断。MA 是可治愈的，且预后良好，因为大多数患者在手术后没有癫痫发作和复发。
