MRI未见异常的库欣病的诊断和处理

Diagnosis and management of Cushing's disease with normal MR finding

目的 探讨MRI未见异常的库欣病的诊断和处理原则。方法 回顾性分析 36例MRI未见异常的库欣病的临床资料 ,除 1例Nelson综合征外 ,其余 35例均有典型的皮质醇增多症的表现。内分泌学检查符合库欣病 31例 ,不典型 5例。 36例均行经蝶窦垂体探查术。随访时间 0 5～ 4年 ,平均 1 5年。结果 36例中病理为垂体ACTH腺瘤 2 7例 ,垂体ACTH细胞增生 4例 ,未见肿瘤或增生 5例。结合内分泌检查及临床表现 ,全组治愈率为 6 7% ,缓解率为 19%。结论 对于MRI未见异常的库欣病 ,如果临床和内分泌学典型 ,可行经蝶窦垂体探查术 ;对内分泌学检查不典型而临床排除异位ACTH腺瘤的患者有手术探查的相对适应证。

Objective To discuss the diagnosis and managmental principle of Cushings disease with normal MR finding. Methods Clinical data of 36 cases of Cushings disease with normal MR finding were analyzed retrospectively.Besides one Nelson syndrome case,the other 35 patients had typical clinical manifestations of hypercorticoidism.Endocrinological assessment supported the diagnosis of Cushings disease in 31,the other 5 cases had atypical endocrine data.All patients were treated by transsphenoidal pituitary exploration. The follow up period ranged from half a year to 4 years(average 1 5 yrs). Results Pathological examination confirmed pituitary ACTH adenoma in 27,pituitary ACTH cell hyperplasia in 4,no evidence of adenoma or hyperplasia in 5 cases.Combined with endocrinological results and clinical features,the curative and remissive ratio was 67% and 19% respectively in our study. Conclusions For patients of Cushings disease with normal MR finding, transsphenoidal pituitary exploration could be used if the patient have typital clinical and endocrinological features.Patients with atypical endocrine data have relative surgical indication so if ectopic ACTH secreting adenoma could be excluded.