第三脑室脊索样胶质瘤一例报道并文献复习

Chordoid glioma of the third ventricle:a case report and review of literature

目的探讨第三脑室脊索样胶质瘤的临床病理学特点、免疫组织化学特征及其相关鉴别诊断,以期提高临床医师对第三脑室脊索样胶质瘤的认识。方法对1例第三脑室脊索样胶质瘤患者的临床相关资料、组织形态学、免疫组织化学标记及其鉴别诊断进行探讨,并复习相关文献。结果患者女,53岁。主要症状为视物模糊,双侧鼻侧视野部分缺损伴头痛、头晕。CT及MRI检查提示肿瘤位于鞍上。光学显微镜下见簇状、条索状上皮样细胞埋在空泡状黏液样基质中,肿瘤周边间质内特征性地出现淋巴细胞、浆细胞浸润。免疫组织化学检查示,肿瘤细胞弥漫性表达神经胶质原纤维酸性蛋白(GFAP)、波形蛋白(Vimentin),灶性表达上皮细胞膜抗原(EMA),增殖细胞标记抗原(Ki67)表达<1%。结论第三脑室脊索样胶质瘤是一种罕见的中枢神经系统肿瘤,其组织学发生尚未完全明确。其诊断主要依靠临床资料、组织形态学及免疫组织化学检查。

Objective To study the clinicopathological features,immunophenotypes and differential diagnosis of chordoid glioma of the third ventricle（CGTV）,so as to provide more information for clinical doctors.Methods The clinical data,morphology,immunohistochemical labeling and differential diagnosis of a CGTV patient were discussed,and the literatures were reviewed.Results The patients was a 53 years old female,with the main symptoms including visual decrease,deficit of visual field of the nasal side,and headache.CT and MRI showed that the tumor was located at the suprasella turcica.Microscopically,the tumor was composed of epithelioid cells in a mucinous background with abundant esinophilic cytoplasm in the nests and strands.There was a mixed chronic inflammatory infiltrate of lymphocytes and plasma cells around the tumor.Necrosis and endothelial proliferation were not identified.Tumor cells were positively stained for glial fibrillary acidic protein（GFAP）and vimentin;there were also focus staining of epithelial membrane antigen（EMA）.Less than 1% of the cells were positive of Ki-67.Conclusions CGTV is a rare tumor of the central nervous system;it can be differentiated from other tumors by the clinical and histopathological features and immunohistochemical staining results.