乳头状胶质神经元肿瘤临床病理特征

Clinicopathological Characterization of Papillary Glioneuronal Tumor:Case Report and Literature Review

背景与目的:报道1例罕见的乳头状胶质神经元肿瘤(papillary glioneuronal tumor,PGNT),结合文献探讨其临床病理学特征。方法:对1例PGNT的临床表现、影像学特征、病理形态改变和免疫组化标记进行观察分析。结果:患者16岁,临床表现为头痛和偶发癫痫发作。MRI检查见右额顶叶脑实质内有边缘清楚的囊性病灶,囊壁薄,囊腔面见微小突起,囊内充有液体。组织学检查见肿瘤由小圆形细胞组成,类似脑室中央神经瘤瘤细胞,并见星形胶质细胞成分,类似毛细胞性星形细胞瘤样结构。囊腔面瘤细胞排列成特征性的假乳头样结构,中心为血管,表面为单层或多层胶质纤维酸性蛋白(GFAP)阳性的星形细胞。肿瘤与正常脑组织分界清楚,无包膜,靠近正常组织的瘤细胞成片排列,局部有中央神经细胞瘤的无核区结构,瘤细胞对神经元免疫组化Syn、NSE、CgA标记呈阳性反应。肿瘤中见个别中型神经节细胞分化细胞,未见坏死和核分裂相。患者术后追踪8个月,未见肿瘤复发。结论:PGNT是一种新鉴定的神经元-胶质混合性肿瘤实体,属良性脑肿瘤,预后良好。

BACKGROUND ＆ OBJECTIVE： To report 1 case of papillary glioneuronal tumor （PGNT） and to describe its clinicopathological features with review of the literatures. METHODS： The clinic features, pathological morphological changes, immunohistochemical label and image findings of one case of PGNT were observed and analyzed. RESULTS： The patient was 16 years old and presented with intermittent headaches and seizures. Magnetic resonance imaging showed a cystic lesion in right parietal-temporal lobe with small mural projects and some fluid in the cyst. Histopatholigical examination revealed that the tumor composed of small round ceils similar to central neurocytoma and glioma cells similar to pilocytic astrocytoma. The tumor cells faced to cystic lumen arranged as papillary-like with one or more layer ceils over the central vessels. These cells were GFAP positive astrocytes. The tumor had a clear margin but was not capsulated. The cells are small round ones and they formed solid sheets with some undefined non nuclear zones. Tumor cells were positive for neuronal immune labels but with no ganglion differentiation. Neither mitoses nor necrosis was seen in the tumor. The patient has been followed up for eight months without evidence of tumor recurrence. CONCLUSIONS： PGNT is a new identified variant of mixed neuronal glial neoplasm. It is a benign tumor and has a favorable outcome.