Department of Pathology, the First Affiliated Hospital, Sun Yat-sen University, 58 Zhongshan Road II, Guangzhou 510080, China.
Diagn Pathol. 2010 Jun 18;5:39. doi: 10.1186/1746-1596-5-39.
Meningioangiomatosis is a rare hamartomatous lesion or meningiovascular malformation in brain. In extremely rare condition, meningioma may occur together with meningioangiomatosis, and only 19 cases have been described in English literature until now. We now report a case of meningioangiomatosis-associated meningioma with atypical and clear cell variant. A 34-year-old man presented a 3-month history of progressive numbness and weakness of his left lower extremity. He had no stigmata of neurofibromatosis type 2. Magnetic resonance imaging (MRI) revealed multifocal lesions in the right frontoparietal lobe. The lesions were totally removed. Microscopically, parts of lesions were atypical and clear cell meningioma corresponding to WHO grade II. The adjacent brain parenchyma showed the histological features of meningioangiomatosis. Neoplastic cells in atypical meningioma area were immunoreactive to epithelial membrane antigen (EMA) with high MIB-1 index of up to 20%. However, the spindle cells in meningioangiomatosis area were negative for EMA with low MIB-1 index of up to 1%. The diagnosis of atypical meningioma associated with sporadic meningioangiomatosis was made. To our knowledge, this is the first case of a meningioangiomatosis-associated meningioma with atypical and clear cell variant component to be described. The patient had been followed-up for 11 months without adjuvant radiotherapy or chemotherapy. No tumor recurrence was found during this period. Meningioangiomatosis-associated meningioma is more likely to occur in younger patients and histologically to mimic parenchymal invasion of brain. We suggest that postoperative radiotherapy or chemotherapy should be given careful consideration to avoid over-treatment due to erroneously interpret as malignant meningioma.
脑脊膜血管瘤病是一种罕见的脑部错构性病变或脑脊膜血管畸形。在极罕见的情况下，脑膜瘤可能与脑脊膜血管瘤病同时发生，迄今为止，英文文献中仅描述了 19 例。我们现在报告一例伴有非典型性和透明细胞型脑膜瘤的脑脊膜血管瘤病相关脑膜瘤。一名 34 岁男性，表现为进行性左下肢麻木和无力 3 个月。他没有神经纤维瘤病 2 型的特征性体征。磁共振成像（MRI）显示右额顶叶多发病灶。这些病变被完全切除。显微镜下，部分病变为非典型性和透明细胞脑膜瘤，对应 WHO 分级 II 级。邻近的脑实质显示出脑脊膜血管瘤病的组织学特征。非典型脑膜瘤区域的肿瘤细胞对上皮膜抗原（EMA）呈免疫反应性，MIB-1 指数高达 20%。然而，脑脊膜血管瘤病区域的梭形细胞对 EMA 呈阴性，MIB-1 指数低至 1%。诊断为非典型脑膜瘤伴散发性脑脊膜血管瘤病。据我们所知，这是首例伴有非典型性和透明细胞型脑膜瘤的脑脊膜血管瘤病相关脑膜瘤病例。患者接受了 11 个月的随访，未接受辅助放疗或化疗。在此期间未发现肿瘤复发。脑脊膜血管瘤病相关脑膜瘤更可能发生在年轻患者中，且组织学上可能模拟脑实质侵犯。我们建议术后放疗或化疗应慎重考虑，以避免因错误地解释为恶性脑膜瘤而过度治疗。
