空泡蝶鞍合并垂体促肾上腺皮质激素瘤病例报道并文献复习

Empty Sella Combined with ACTH-producing Pituitary Tumor: a Case Report and Literature Review

空泡蝶鞍与垂体促肾上腺皮质激素(ACTH)瘤两种疾病并发较为罕见,国内外报道较少。本文报道1例患者影像学检查不能确诊又高度怀疑垂体ACTH瘤,经双侧岩下窦静脉取血(BIPSS)联合去氨加压素(DDAVP)试验,双侧岩下窦ACTH水平明显高于外周静脉,提示垂体ACTH瘤存在。术后病理显示垂体腺瘤,确诊为空泡蝶鞍合并库欣病。此患者的诊断过程提示,BIPSS联合DDAVP试验可用于指导影像学检查无明确结果的库欣病患者的鉴别诊断和指导治疗。

Empty sella combined with ACTH-producing pituitary tumor is a rare disease,and it has been barely reported. In this article,we reported the diagnosis of one case of empty sella combined with Cushing＇ s disease. At first,the patient was highly suspected of having ACTH-producing pituitary tumor but could not be diagnosed confirmedly by the results of imaging examination only. The results of bilateral inferior petrosal sinus sampling（ BIPSS） test and DDAVP test found that the ACTH levels in bilateral inferior petrosal sinus were significantly higher than those in the peripheral vein,which demonstrated that the patient might have ACTH-producing pituitary tumor. Postoperative histological examination proved the presence of ACTH-producing pituitary tumor. The patient was confirmedly diagnosed with empty sella combined with Cushing＇s disease. Therefore,when imaging examination is not confirmedly reliable for identifying Cushing＇s disease,BIPSS test combined with DDAVP test can be used as a reference for the differential diagnosis and treatment of the disease.