Pulmonary carcinoid (PC) is a rare low-grade malignant neuroendocrine tumor. Unlike other neuroendocrine tumors of the digestive tract, PC seldom causes hormone-related neuroendocrine symptoms, and the diagnosis mainly depends on the biopsy of the lesion. Besides, the immunohistochemical staining technique is an important supplement and a grading standard for pathological diagnosis. Surgery is the first choice for the treatment of early PC. However, the therapy of advanced PC which is the main problem perplexing most of clinicians is controversial and still facing severe challenges. Therefore, we will discuss the current progress of the diagnosis and therapy of advanced PC.
Objective To investigate the clinical features, diagnosis, treatment and prognosis of ectopic adrenocorticotropic hormone (ACTH) syndrome caused by bronchopulmonary carcinoid. Methods Twenty patients (13 males and 7 females) with ectopic ACTH syndrome caused by bronchopulmonary carcinoid confirmed by surgical pathology were selected from March 2007 to May 2016 in our hospital. We collected and arranged the important clinical data of 20 patients and analyzed carefully. Results The mean age of 20 patients including 14 patients of typical carcinoid and 6 patients of atypical carcinoid was 38.85±15.31 years ranged 13-70 years. All patients had the typical clinical manifestation of Cushing's syndrome. All the thoracic lesions were located by thoracic computed tomography (CT) eventually. The concentration of serum cortisol and ACTH significantly decreased after surgery. Postoperative complications in 3 patients (15.00%) were severe pulmonary infection. Eighteen patients including 13 patients of typical carcinoid and 5 patients of atypical carcinoid were followed up. The median time of follow-up was 31.5 (12-122) months. There were 7 patients of stage Ⅰa, 1 patient of stage Ⅰb, 2 patients of stage Ⅱa and 8 patients of stage Ⅲa in the patients followed up. One patient underwent postoperative radiotherapy, and 4 patients with postoperative chemotherapy. During the follow-up period, one patient relapsed and there was no death. Conclusion Ectopic ACTH syndrome caused by bronchopulmonary carcinoid is a very rare disease. It is every difficult for clinicians to make early diagnosis and draw up plans of treatment without multidisciplinary collaboration. Chest CT is an important method of finding lesions and assessing the viability of surgery. Surgical treatment in early stage will be effective for this disease. And almost all of patients can achieve long-term survival after complete resection of tumors early.