Clinical Diagnosis And Treatment Of Ectopic ACTH Syndrome Caused By Bronchopulmonary Carcinoid

ObjectiveTo investigate the clinical features,diagnosis,treatment and prognosis of ectopic adrenocorticotropic hormone syndrome caused by bronchopulmonary carcinoid.MethodsTwenty patients with ectopic ACTH syndrome caused by bronchopulmonary carcinoma confirmed by surgical pathology were selected from March 2007 to May 2016 in Peking Union Medical College Hospital,including 13 males and 7 females.Collected and arranged the important clinical data of 20 patients and analyzed carefully.ResultsThe mean age of 20 patients was 38.85±15.31 years old(range:13?70 years old)and the median duration of disease was 15 months(range:1?156 months).All patients had the typical clinical manifestation of Cushing's syndrome.All the thoracic sources were located by thoracic computed tomography(CT)eventually.The concentration of serumcortisol and ACTH was significantly decreased after surgery.Postoperative complications in 3 cases(15%)were severe pulmonary infection.18 patients including 13 cases of typical carcinoid and 5 cases of atypical carcinoid were followed up.The median time of follow-up was 31.5 months(range:12?122 months).There are 7 cases of ?a,1 case of ?b,2 cases of stage ?a and 8 cases of stage ?a in patients followed up.1 patient underwent postoperative radiotherapy,and Postoperative chemotherapy were performed in 3 cases.During the follow-up period,one patient relapsed and no death.The differences of Ki67,postoperative serum cortisol,and tumer size between typical carcinoid and atypical carcinoid were statistically significant.Meanwhile,the differences of turner size between smoker and non-smoker,and the duration of disease between patients with lymph node metastasis or not were statistically significant.Moreover,the level of serum ACTH was positively correlated with age and negatively correlated with serum potassium.Conclusions1.Bronchopulmonary carcinoma caused by ectopic ACTH syndrome is a very rare disease.It is every difficult for clinicians to make early diagnosis and draw up plans of treatment without multidisciplinary collaboration;2.Chest CT is an important method of finding lesions and assessing the viability of surgery;3.Surgical treatment in early stage will be effective for this disease.And almost all of patients could be long-term survival after complete resection of tumor early;4.Do not recommend with adjuvant therapy routinely for PTC or PAC patients with complete tumor resection,whether there are regional lymph nodes metastases or not;5.Pathological type and tumor stage may be factors influencing the prognosis,which,however,need to be confirmed by accumulating more data;6.Older women may be more prone to severe hypokalemia,and postoperative serum cortisol may be one of the factors associated with prognosis.