间变型脑膜瘤临床病理分析

Anaplastic Meningioma:A Clinicopathologic Study

目的探讨间变型脑膜瘤(anaplastic meningioma,AM)的临床病理特征及病理诊断。方法收集17例AM,分析其临床表现、病理特点、免疫组织化学及分子遗传学,并复习文献。结果患者年龄46~84岁,女性6例、男性11例,6例继发于低级别脑膜瘤、11例为原发性AM,均行手术治疗,9例行辅助治疗。镜下瘤细胞密集,瘤细胞多形性、异型性显著,核分裂象活跃,1例伴腺癌样化生。免疫组化瘤细胞弥漫表达波形蛋白(vimentin),不同程度表达上皮细胞膜抗原(EMA)、生长抑素受体(SSTR2),腺癌样化生区表达细胞角蛋白。1例行NGS检测示NF2缺失、PTEN缺失,2例行ARMS法示TERT启动子野生型。随访1~46个月,11例原发性AM中5例于7~25个月复发,转移2例,死亡3例。结论AM是一种少见的恶性肿瘤,组织形态多样,出现腺癌样化生时,需与腺癌转移至脑膜瘤、滑膜肉瘤等鉴别。

Objective To study the clinicopathological features and diagnosis of anaplastic meningiomas(AM).Methods 17 AMs were enrolled to evaluate the clinical presentations,pathological features,immunohistochemical and molecular genetics,and related literatures were reviewed.Results The patients included 6 females and 11 males,ranged from 46 to 84 years old.6 cases were secondary to low-grade meningiomas,11 cases were primary AMs.All patients accepted surgery,9 of them accepted additional therapy.Microscopically,the tumors presented cellularity,obvious pleomorphism and atypia,and active mitosis.There was focal adenocarcinoma-like metaplasia in 1 case.Immunohistochemistry showed diffuse positivity for vimentin,while EMA and SSTR2 were positive variably.Adenocarcinoma-like metaplasia showed positivity for CKs.NGS testing in 1 case showed deletion of NF2 and PTEN,and ARMS testing in 2 cases showed wild-type TERT promoter.During the follow-up of 1 to 46 months,5 cases of primary AM recurred in 7 to 25 months,there were 2 metastases and 3 deaths.Conclusions AM is a rare malignant tumor with variable morphology,when adenocarcinoma-like metaplasia occurs,it should be differentiated from adenocarcinoma metastasis to meningioma and synovial sarcoma,etc.