Kong Xiangyi, Wang Renzhi, Yang Yi, Wu Huanwen, Su Changbao, Ma Wenbin, Li Yongning, Xing Bing, Lian Wei, Xu Zhiqin, Yao Yong, Ren Zuyuan
From the Department of Neurosurgery (XK, RW, YY, CS, WM, YL, BX, WL, ZX, YY, ZR), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences; and Department of Pathology (HW), Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan Hutong of Dongcheng District, Beijing, P. R. China.
Medicine (Baltimore). 2015 Jul;94(28):e1099. doi: 10.1097/MD.0000000000001099.
Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease of the pituitary that commonly presents with enlargement of the pituitary gland. Clinically and radiologically, IGH is a rare sellar entity easily to be misdiagnosed as a pituitary adenoma. Through such a case, we aim to present this rarity and emphasize the importance to correctly diagnose confusing pituitary lesions comprehensively by clinical presentations, radiological signs, and biopsy. We present an uncommon case of IGH in a 19-year-old man. The patient was admitted to the hospital with severe headache, vomiting, and vision's sharp decline. Magnetic resonance imaging showed a sellar lesion with obvious cystic change and ring enhancement. The disease course including diagnosis and treatment was presented and analyzed in detail. The pertinent literature is reviewed regarding this uncommon entity. The patient underwent surgical exploration and partial resection via the transsphenoidal approach. The pathologic findings suggested IGH giving no significant evidences of systemic granulomatous disease and venereal disease. Large dose methylprednisolone was then used. The pituitary function recovered, but there was no apparent improvement of his vision. IGH is a rarely occurred inflammatory disease of unknown etiology. It is difficult to diagnose preoperatively and is often misdiagnosed. Although rare, IGH should be kept in mind in terms of differential diagnosis of sellar region lesions.
特发性肉芽肿性垂体炎（IGH）是一种罕见的垂体炎性疾病，通常表现为垂体增大。在临床和影像学上，IGH是一种罕见的鞍区病变，容易被误诊为垂体腺瘤。通过这样一个病例，我们旨在展示这种罕见疾病，并强调通过临床表现、影像学征象和活检全面正确诊断疑难垂体病变的重要性。我们报告一例19岁男性的罕见IGH病例。该患者因严重头痛、呕吐和视力急剧下降入院。磁共振成像显示鞍区病变伴有明显的囊性改变和环形强化。详细介绍并分析了该病例的病程，包括诊断和治疗过程。对关于这种罕见疾病的相关文献进行了综述。患者接受了经蝶窦入路的手术探查和部分切除。病理结果提示为IGH，未发现明显的系统性肉芽肿病和性病证据。随后使用了大剂量甲泼尼龙。垂体功能恢复，但视力无明显改善。IGH是一种病因不明的罕见炎性疾病。术前难以诊断，常被误诊。尽管罕见，但在鞍区病变的鉴别诊断中应考虑到IGH。
