透明细胞型脑膜瘤临床病理分析

目的:探讨透明细胞型脑膜瘤的临床病理特征、诊断及鉴别诊断。方法:回顾性分析6例透明细胞型脑膜瘤患者临床及影像学资料、组织学形态及免疫组化结果，并进行随访。结果:患者6例，男性2例，女性4例，手术时年龄14～39岁（平均年龄:26岁）。发生部位分别为桥小脑角区5例（左侧1例，右侧4例，其中1例为多发，包括右侧桥小脑角区及双额叶间大脑镰旁），骶尾部1例。临床症状与肿瘤部位相关。肿瘤最大直径约3.3~17.2 cm。手术方式分别是3例完全切除，2例次全切除，1例为穿刺活检。病理学检查示瘤细胞呈片状排列,中间交织着透明变性的块状胶原，细胞呈多边形,胞质透明,胞核居中。免疫组化显示EMA（6/6）、PR（5/6）、Vimentin（6/6）阳性,GFAP、S-100蛋白、CK均为阴性,特殊染色显示PAS(+),D-PAS（-）。Ki67标记指数2%~10%。3例获得随访资料，随访时间6~25个月，1例（例2）完全切除术后死亡，1例（例3）次全切除术后进行放射治疗，现带病生存，1例（例6）完全切除术后无病生存。结论:透明细胞型脑膜瘤是一种罕见的脑膜瘤类型，诊断时依靠形态学结合免疫组化染色，需与胞浆透亮的脑肿瘤鉴别。该类型肿瘤好发于年轻人，复发率高，建议进行影像学监测。

Clinical pathological study of clear cell meningioma

Objective:To investigate the clinicopathologic features，diagnosis and differential diagnosis of clear cell （CCM）.Methods:6 cases of clear cell meningioma were studied by clinicopathology，imaging，and immunohistochemistry，3 cases were followed up.Results:There were 6 patients，2 males and 4 females.The age at operation was 14~39 years (mean:26 years).There were 5 cases in the cerebellopontine angle region (1 case on the left side，4 cases on the right side) and 1 case in the sacrococcygeal region.The clinical symptoms are related to the site of the tumor.The maximum diameter of the tumor was about 3.3~17.2 cm.The operation was performed in 5 cases，total resection in 3 cases，subtotal resection in 2 cases.1 case was diagnosed by fine-needle aspiration of tumor.Histologically，the tumor cells were sheeting architecture and polygonal cells with clear cytoplasm and prominent blocky collagen.Immunohistochemically，the tumor were positive for EMA (6/6)，PR(5/6)，Vimentin (6/6)，and negative for GFAP，S-100，CK.All 6 cases showed positive PAS stain.The Ki67 labeling index was 2%~10%.3 cases were followed-up for 6~25 months.Conclusion:CCM is a rare subtype of meningioma.Its diagnosis is based on morphology and immunohistochemical staining.It should be differentiated from other brain tumors with clear cytoplasm.CCM tend to affect younger patients with high recurrence rate.It is recommended to monitor the image.