神经垂体颗粒细胞瘤的临床病理分析

Clinical pathological analysis of neurohypophyseal granular cell tumor

目的探讨神经垂体颗粒细胞瘤(GCT)的临床与病理学特征,以提高对此罕见病的诊断及鉴别诊断。方法回顾性分析3例神经垂体GCT的临床病理资料,并复习相关文献。结果 3例患者中男1例,女2例,年龄41~66岁。肿瘤由致密排列的多角形或圆形细胞构成,胞浆丰富,略嗜酸呈细颗粒状,无坏死及核分裂象。瘤细胞表达酸性钙链接蛋白S-100(3/3)、细胞表面糖蛋白CD56(2/3)、波形蛋白(Vimentin,2/3)、胶质纤维酸性蛋白(GFAP,1/3),增殖细胞核抗原Ki-67约1%~5%。肿瘤细胞过碘酸雪夫氏(PAS)染色(+)。随访3例患者,均存活。结论神经垂体GCT非常罕见,对于鞍区发生的肿瘤,术前应考虑到神经垂体GCT的可能,以免误诊和延误治疗。

Objective The clinical and pathological features of granular cell tumor （GCT） of neurohypophysis were investigated to improve the diagnosis and differential diagnosis.Methods Three cases of neurohypophyseal GCTs were retrieved.Their clinicopathologic and immunohistochemical features were studied,and the related literature was also reviewed.Results Three patients included 1 male and 2 female patients aged from 41 to 66 years old.Histologically,the tumor was composed of round or polygonal cells with abundant granular eosinophilic cytoplasm.No necrosis and significant mitotic figures were identified.Immunohistochemically,the tumor cells showed strongly positive for S-100 （3/3）,CD56 （2/3） and Vimentin （2/3）,while weak or focal positive for glial fibrillary acidic protein （GFAP,1/3）,and Ki-67 proliferation index was low （1％ ～ 5％）.Its abundant cytoplasm contained granules positive for diastase-resistant periodic acid-schiff reaction.The follow-up showed that 3 cases survived.Conclusion Although extremely rare,the granular cell tumors of neurohypophysis have to be considered in the differential diagnosis of suprasellar masses to avoid the misleading interpretation and consequent wrong therapeutic management.