探讨横纹肌样脑膜瘤的临床病理特点和鉴别诊断.docxVIP

探讨横纹肌样脑膜瘤的临床病理特点和鉴别诊断 南华大学附属第一医院湖南衡阳421001 【摘 要】目的探讨横纹肌样脑膜瘤(RM)的临床病理特点和鉴别诊断。方法： 选取2010年一 2015年间2例病例。患者发病年龄分别为6、16岁，男女各1 例。分析RM的临床表现、组织形态学及免疫表型，并复习相关文献。结果：送 检标木均为手术切除的肿瘤组织，总体积约为6—28cm。肿物无包膜，灰白至灰 褐色，部分区域旱鱼肉状，切面实性，质软，其中1例局灶质韧，1例可见出血 及坏死区域。光镜下肿瘤细胞呈横纹肌样特点，嗜酸性胞浆及偏位核。免疫组织 化学染色显示肿瘤细胞显示Vim弥漫阳性，EMA弥漫或灶状阳性，INI-1核阳性， p53弱阳性,Ki67阳性表达率大于30%；而GFAP、HMB45和PR阴性，S—100 和CK (AE1/AE3)表达不一，或弥漫阳性，或阴性表达；其余抗体偶见弱表达。 结论：横纹肌样脑膜瘤非常罕见，具有横纹肌样的细胞形态学特点，免疫组化是 正确诊断横纹肌样脑膜瘤的重要依据。 【关键词】横纹肌样脑膜瘤；病理；诊断 [Abstract] Objective： To investigate rhabdoid meningioma (RM) clinical pathological characteristics and differe ntial diag no sis. Methods： 2 cases of 2015 cases were selected in 2010. The age of on set was 6, 16 years old, 1 cases were male and female. To an a lyze the clin ical man ifestati ons, morphology and immune phe no type of RM, and review the related literatures. Results： the specimens were resected tumor tissue, the total volume of about 6 ? 28cm. The tumor was not enveloped, gray to gray brown, some areas of dry fish, plane of solid, soft, of which 1 cases of focal ductile, 1 cases of bleeding and necrosis of the area. Under the light microscope rhabdoid tumor cells, eosinophilic cytoplasm and partial nuclear. Immunohistochemical staining showed that the tumor cells showed VIM diffuse positive EMA diffusely or focally positive ini-1 nuclei positive for p53 weak positive, the positive expression of Ki67 rate of more than 30%； and GFAP, HMB45 and PR n egative, s ? 100 a nd CK (AE1 / AE3) expressio n is no t a, or diffuse a nd positive or n egative expressi on ； the rest of the an tibody occasi on ally weakly expressed. Conclusion： rhabdoid meningioma is very rare, with rhabdoid cell morphological characteristics and immuno histochemical is an importa nt basis for the correct diagnosis of rhabdoid meningioma. [keyword]： rhabdoid meningioma； Pathology； diagnosis 横纹肌样脑膜瘤(rhabdoid meningioma, RM)是脑膜瘤中较为少见的 病理亚型，由Perry等⑴ 在1988年首次明确提出，并将其定义为具有横纹肌样 细胞的脑膜瘤，强调其具有较高的侵袭性。RM具有很强的增殖活性，2007年 世界卫生组织(World Health