散发型脑膜血管瘤病7例临床病理分析

Sporadic Meningioangiomatosis: A Clinicopathological Analysis of Seven Cases

目的探讨脑膜血管瘤病(meningioangiomatosis,MA)的临床病理特征、诊断及鉴别诊断特点。方法回顾性分析7例MA患者的临床影像资料、组织学及免疫组织化学结果等,并复习文献。结果 7例MA中,男2例,女5例。年龄4~56岁,平均17.86岁。5例以癫痫发作为首发症状。影像学上,6例表现为占位性病变,但缺乏特异性。主要组织病理学表现是大脑皮质浅层血管及血管周围梭形细胞明显增殖。血管不同程度的玻璃样变、钙化。梭形细胞显示了一些脑膜上皮细胞的组织学特征,细胞核呈圆形到伸长形,胞质嗜伊红,无明显异型性及核分裂。病灶内陷入的神经胶质反应性增生,神经元发生退行性变伴神经原纤维缠结形成。免疫组织化学显示所有病例血管及血管周围梭形细胞vimentin阳性。3例梭形细胞成分EMA局灶阳性、PR散在细胞阳性。病灶内细胞增殖指数低。7例均行肿瘤全切术,术后均未行放化疗。随访时间1~29月,均无复发,所有病例无瘤生存。结论 MA是一种罕见的生长缓慢的颅内良性病变。结合临床影像检查、组织病理学及免疫组织化学结果等有利于诊断和鉴别诊断。MA患者经手术治疗后预后较好。

Objective To investigate the clinicopathological characteristics, diagnosis and differential diagnosis of meningioangiomatosis(MA). Methods The clinical manifestations, radiological, histopathological and immunohistochemical features were analyzed retrospectively in 7 MA patients. Related literatures were reviewed. Results Of 7 patients, 2 were males and 5 were females. The age ranged from 4 to 56 years old with a mean age of 17.86 years old. Five patients presented seizure as the initial symptom. Six cases appeared as mass lesion on neuroimaging, but the radiographic findings of MA may show a variety of features. All lesions, usually involved the cerebral cortex, were composed of two main elements, proliferative small blood vessels and perivascular spindle cells. These blood vessels showed variable degrees of hyalinization and calcification. These perivascular spindle cells exhibited some histological features, similar to meningothelial cells. The nuclei were round to elongated, with bland chromatin. The cell membranes appeared fuzzy or indistinct, and eosinophilic cytoplasm. There was no obvious atypia or mitoses. There were reactive gliosis and microcalcification in entrapped cortex. Degenerative changes of neuron were sometimes found, such as neurofibrillary tangles. Immunohistochemically, the proliferative perivascular spindle cells were positive for vimentin in all cases, and were focal positive for EMA and scattered immunoreactive for PR in 3 cases. MIB1 labeling index(MIB1 LI) was low. All patients received surgical treatment without chemotherapy or radiotherapy postoperatively. No recurrence was confirmed during follow-up from 1 to 29 months. All cases were disease-free survival. Conclusion MA is a rare slow-growing intracranial lesion. Histopathological and immunohistochemical features combined with radiological are helpful in diagnosis. MA patients have a good prognosis after surgical treatment.