异位促肾上腺皮质激素垂体腺瘤三例并文献复习

3 cases of ectopic ACTH pituitary adenoma and literature review

目的对3例异位促肾上腺皮质激素垂体腺瘤（EAPA）患者的临床资料进行分析，初步探讨EAPA的诊治方法。方法回顾性分析1994年至今北京协和医院就诊的库欣病1024例，其中3例为经手术病理证实的EAPA，女2例，男1例，年龄19～22岁。结合文献对其临床资料进行分析。结果EAPA位于鞍上（2例）和左侧海绵窦（1例）。1例大剂量地塞米松抑制试验不被抑制，2例接受了岩下窦静脉取血（IPSS），1例（鞍上占位）岩下窦与外周血的促肾上腺皮质激素（ACTH）比值为10．26；而另1例（左侧海绵窦占位）为〈2。3例患者术后血ACTH及尿皮质醇水平均明显下降。结论EAPA是库欣病的罕见病因，诊断及手术均存在较大难度，IPSS对EAPA有一定诊断价值。

Objective To evaluate the clinical and laboratory features of 3 cases of ectopic ACTH pituitary adenoma （EAPA）. Methods 3 cases of EAPA confirmed by surgery and pathological examination have been retrospectively investigated in Peking Union Medical College Hospital since 1994. There were 2 female and 1 male patients with age ranging between 17 - 22y. The relevant literatures of EAPA were reviewed. Results The level of plasma ACTH was 126 - 207 pg/ml. The result of high dose dexamethasone suppression test was not suppressed in 1 case. The localization of EAPA tumor was suprasellar in 2 cases and left cavernous sinus in 1 case. The results of inferior petrosal sinus sampling （IPSS） showed the ratio of inferior petrosal sinus （IPS） ： Peripheral （P） of plasma ACTH was 10. 26 for 1 case with suprasellar EAPA and 〈 2 for the other 1 case with left cavernous sinus EAPA. The pathological examination confirmed the diagnosis of EAPA with positive immunohistochemical staining of ACTH for all 3 cases after operation. The level of plasma ACTH and urine free cortisol decreased obviously after operation. Conclusions EAPA should be considered in the differential diagnosis for all patients with Cushing ＂s syndrome. The diagnosis and treatment of EAPA tumor are both difficult to carry out. IPSS may be helpful for the diagnosis of EAPA.