散发型脑膜血管瘤病临床病理学分析

Sporadic meningioangiomatosis:a clinicopathological analysis

目的探讨散发型脑膜血管瘤病(meningioangiomatosis,MA)的临床病理学特征、诊断及预后,分析血管周围梭形细胞的来源。方法收集2012年6月至2020年3月首都医科大学宣武医院和福建医科大学附属第一医院诊断的散发型MA病例17例,分析患者的临床资料、影像学特征、病理学特点及预后,通过相关免疫组织化学染色分析血管周围梭形细胞来源。结果 17例散发型MA患者,男性9例,女性8例,年龄3~56岁,以难治性癫痫为首发症状者13例。影像学显示病灶均位于大脑皮质,为单发肿块。组织学表现为大脑皮质浅层见显著增生的血管,并于血管周围见梭形细胞增生,梭形细胞形态温和,无明显异型性,未见明显核分裂象及坏死,其中4例合并过渡型脑膜瘤。免疫组织化学染色显示血管周围增生的梭形细胞波形蛋白均阳性,12例SSTR2阳性,11例上皮细胞膜抗原阳性,Ki-67阳性指数较低,8例AT8染色显示退行性变的神经元轴突及神经原纤维缠结形成。随访时间为1~93个月,17例病例术后均无癫痫发作、无肿瘤复发。结论 MA是一种颅内罕见的良性错构性瘤样病变,可合并脑膜瘤。血管周围增生的梭形细胞倾向于脑膜上皮细胞来源,病变皮质可发生退行性变。手术切除患者预后良好。

Objective To analyze the clinicopathological characteristics,diagnosis and prognosis of meningioangiomatosis(MA),and to investige the possible origion of spindle cells.Methods Seventeen cases of MA were collected at Xuanwu Hospital of Capital Medical University and the First Affiliated Hospital of Fujian Medical University,from June 2012 to March 2020.The clinical manifestations,radiologic,histopathologic,immunohistochemical features and patients′outcome were analyzed.The presumed origin of spindle cells was evaluated by immunohistochemical staining.Results Of the 17 patients,9 were males and 8 were females.The age ranged from 3 to 56 years old.Thirteen patients presented with seizure as the initial symptom.The lesions were solitary and located in the cerebral cortex.Histopathologically,there were proliferation of small blood vessels and perivascular spindle cells in the cerebral cortex.The spindle cells had no obvious atypia,mitoses and necrosis.Four cases were combined with transitional meningioma.Immunohistochemically,the proliferative perivascular spindle cells were positive for vimentin in all cases,and focally positive for EMA and SSTR2.Ki-67 proliferation index was low.Neurofibrillary tangles were demonstrated by AT8.All 17 patients received surgical treatment and were followed up for one to 93 months.None had seizure attacks or tumor recurrence.Conclusions MA is a rare slow-growing intracranial lesion,and the perivascular spindle cells could be derived from meningothelial cells,and MA is often associated with degeneration of the cerebral cortex and meningioma.The patients have good prognosis after surgical treatment.