Abstract: Objective： To investigation the diagnosis and treatment of ectopic adrenocorticotrophic hormone (ACTH) syndrome. Methods: The clinical characters of 57 cases of ecotopic ACTH syndrome from Jan. 1996 to Dec. 2016 were collected and analyzed. The 57 cases included 32 males and 25 females. The age ranged from 11 to 68 years ( average 32 years). ACTH levels significantly increased from 16.5 to 365.6 pmol/L, with average 77.6 pmol/L (normal range ＜10.1 pmol/L). The pituitary MRI did not found lesions. The CT showed that their bilateral adrenal glands diffused small nodular changes or nodular hyperplasia. The 57 cases were divided into 3 groups according to different treatment options. In the study, 25 ectopic ACTH syndrome cases (44%) were group A, without identified source of ectopic hormone, were treated with bilateral or unilateral adrenalectomy due to the severity of the disease and difficulty of operation. Group B was composed of 16 cases (28%) diagnosed as ectopic ACTH syndrome by finding ectopic ACTH tumors and surgical resection. Group C included 16 cases (28%) with nonsurgical therapy. Different treatment results and prognosis were analyzed. Results: In the study, 40 cases of the 57 had been followed up for 6 months to 10 years. In group A, of the 25 cases with bilateral or unilateral adrenalectomy, 4 died of diabetes and severe pulmonary infection, 18 survived, and 3 were lost to the follow-up, and the survival rate was 81% (18/22). In group B, of the 16 cases with radical tumor resection, 5 died of tumor recurrence 0.5-6.0 years after operation, 3 survived, and 8 were lost to the follow-up, and the survival rate was 37.5% (3/8). In group C, of the 16 non-operation patients, 4 with radiotherapy and chemotherapy died of metastases, diabetes or pulmonary infection, 6 with chemotherapy died of pulmonary infection within 1 year and the others were lost to the follow-up, and the survival rate was 0. Conclusion: Ectopic ACTH syndrome is difficult to treat. Adrenalectomy is effective for the management of ectopic ACTH syndrome, especially for those patients with severe Cushing’s syndrome, but the primary tumor can not be located.

Key words: ACTH syndrome, ectopic, Adrenalectomy, Treatment outcome