神经垂体颗粒细胞瘤临床病理观察

Clinicopathological features of granular cell tumor of the neurohypophysis

目的探讨神经垂体颗粒细胞瘤(GCT)的临床表现、影像学和病理学特征。方法报道1例神经垂体颗粒细胞瘤,进行形态学观察及免疫组化染色,并复习文献。结果患者男性,36岁。间断性头痛10天。头部MRI显示鞍区占位性病变,T1与T2等信号,不均匀增强。手术全切除肿瘤。镜下见肿瘤由密集上皮样细胞构成,胞质丰富,有较多嗜酸性颗粒,细胞核小,细胞异型性不明显,缺乏核分裂。PAS特殊染色可见胞质内富含阳性颗粒。免疫组化:肿瘤细胞CD68、vimentin和TFE-3弥漫性(+),Ki-67阳性指数为3%,CD1a、S-100、TTF-1、Syn和CgA均(-)。结论神经垂体颗粒细胞瘤是鞍区罕见肿瘤,可能起源于神经垂体的特殊神经胶质细胞,明确诊断依靠病理学检查。

Objective To investigate the clinical,neuroimaging and pathological features of granular cell tumor of the neurohypophysis. Methods One case of granular cell tumor of the neurohypophysis was reported,combined with morphological and immunohistochemical investigation,and the relevant literatures were also reviewed. Results A 36-year-old male had caught an intermittent headache for about 10 days. Brain MRI displayed a space-occupying lesion in the saddle area with equal signals in T1WI and T2WI and heterogeneous enhancements. In surgery,the tumor was found to be located in the pituitary area,and was completely removed. In histology,the neoplasm was composed of dense epithelioid cells without obvious atypia. The cytoplasm of the tumor cells was abundant of eosinophilic particles. The nuclei were small and absent of mitoses. PAS staining showed affluent positive particles in the cytoplasm of the neoplasm cells. In immunohistochemistry,CD68,Vimentin,and TFE-3 were diffusely positive,Ki-67 labeling index was about 3%,and CD1 a,S-100,TTF-1,Syn,CgA and so on were negative. Conclusions Granular cell tumor of the neurohypophysis is one kind of rare neoplasms in the saddle area,which is supposed to be originated from specialized glia of neurohypophysis.A clear diagnosis often depends on pathological examination.