脂化细胞胶质母细胞瘤1例报道及文献复习

Clinicopathologic features of lipidized cell glioblastoma:a case report and review of the literature

目的探讨富于脂化细胞胶质母细胞瘤的临床病理诊断及鉴别诊断要点。方法报道1例14岁女孩右颞部皮层下占位性病变的MRI,肿瘤组织学表现及免疫组化结果,并复习相关文献。结果MRI示:右额颞叶大片不规则类圆形T1低、T2高且不均匀的异常信号影,周围呈指状水肿;中线向对侧明显移位,侧脑室受压消失,增强扫描病灶呈不规则环状强化。病理组织学检查:肿瘤主要有两种类型生长方式,主要一类肿瘤细胞胞质脂肪空泡状,细胞核位于一侧;另一部分肿瘤细胞排列呈梭形;两种细胞局部有明显多型性及异型性,可见血管内皮细胞增生及少量坏死。免疫组化:两种类型肿瘤细胞(脂肪样细胞及梭型细胞)均显GFAP()阳性,vimintin(+)阳性,大部分肿瘤细胞p53阳性,Ki-67阳性细胞10%。网织纤维染色梭形细胞成分显明显阳性、脂肪样细胞区阴性。患者术后3个月死亡。结论富于脂化细胞是胶质母细胞瘤的十分少见的新亚型,脂肪样细胞显示强GFAP阳性。主要需与间变型PXA进行鉴别诊断。该病预后结果文献报道不一。

Purpose To investigate the clinicpathologic association and differential diagnosis of lipidized cell glioblastoma. Methods MRI and surgical findings, histopathologic changes and immunohistochemical features of lipidized cell glioblastoma were reported in a 14- year-old girl with a right temporal mass. Results MRI revealed an expanding mass located at the right frontal and temporal lobes with poorly marginated and diffusely infiltrating hemispheric lesions, which was hypointense on T1-WI, and hyperintense with adjacent vasogenic edema on T2-WI/T2-FLAIR. MRI also showed that the middle line markedly shifted to the left, right lateral ventricle deformed and disappeared, and a thick, irregular enhancing ring of neoplastic tissues surrounding a necrotic core. Histopathologically, tumor cells showed 2 types of growth pattern ： most of tumor cells with foamy lipid vacuoles and some spindle cells. There was pleomorphism and anaplasia in both cell types, with foci of necrosis and endothelial proliferation. Both types of the tumor cells were strongly positive for GFAP, vimintin, and p53 staining. In addition, about 10% of the tumor cells were positive for Ki67. The area of foamy lipidized tumor cells was lack of reticulum fiber. The patient died 3 month after operation. Conclusion Lipidized glioblastoma is a very rare subtype of glioblastoma. The tumor cells are strongly positive for GFAP. Differential diagnosis of this tumor mainly includes anaplastic pleomorphic xanthoastrocytoma and the prognosis varies reported in the literature.