伴尿崩症的鞍区颗粒细胞瘤

少年起病、伴尿崩症的颗粒细胞瘤目前国内外尚无文献报道。本文1例患者14岁,男性,以多尿为主要表现,头颅MRI示鞍区实性占位病变,术前垂体功能基本正常,曾考虑淋巴细胞垂体炎和生殖细胞瘤,分别行甲基强的松龙冲击治疗和鞍区放疗均无效果。行经蝶肿瘤切除,病理为颗粒细胞瘤。术后患者病情平稳,因垂体功能低下行替代治疗。

Granular cell tumor of the sellar region associated with diabetes insipidus

Granular cell tumor(GCT) of sellar region is a rare disease which is difficult to be diagnosed before operation. A case of symptomatic granular cell tumor associated with diabetes insipidus was reported in a 14-year-old boy who had suffered from polyuria, polydipsia for over 2 months. No visual abnormality was found. Laboratory test showed mild hyperprolactinemia and normal levels of other pituitary hormones. MRI revealed an sellar mass with suprasellar extension. Transsphenoidal tumor resection was performed and pathological examination showed a GCT. The postoperative course was smooth during 16 months of regular follow-up. The patient received substitution therapy because of hypopituitarism.