Clinical retrospective study of 20 patients with ectopic adrenocorticotropic hormone syndrome caused by bronchopulmonary carcinoid_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

WANG Xuebin , LIU Lei , WANG Guige , ZHANG Jiaqi , CHEN Yeye , CAO Lei , LIU Hongsheng ,  LI Shanqing

Department of Thoracic Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Beijing, 100730, P.R.China;

Corresponding author：

LI Shanqing, Email: lidanqing@pumch.cn

Keywords：

Bronchopulmonary carcinoid; ectopic adrenocorticotropic hormone syndrome; diagnosis; surgery; prognosis

DOI：

10.7507/1007-4848.201802042

Video：

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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.

Citation： WANG Xuebin, LIU Lei, WANG Guige, ZHANG Jiaqi, CHEN Yeye, CAO Lei, LIU Hongsheng, LI Shanqing. Clinical retrospective study of 20 patients with ectopic adrenocorticotropic hormone syndrome caused by bronchopulmonary carcinoid. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(11): 942-948. doi: 10.7507/1007-4848.201802042 Copy

1.	Brown HP. Surgical intervention in extra-uterine pregnancy: observations in the treatment of 109 cases at the pennsylvania hospital. Ann Surg, 1928, 87(4): 581-586.
2.	Bagshawe KD. Hypokalaemia, carcinoma and Cushing’s syndrome. Lancet, 1960, 2(7145): 284-287.
3.	Allott EN, Skelton MO. Increased adrenocortical activity associated with malignant disease. Lancet, 1960, 2(7145): 278-284.
4.	Ilias I, Torpy DJ, Pacak K, et al. Cushing’s syndrome due to ectopic corticotropin secretion: twenty years’ experience at the National Institutes of Health. J Clin Endocrinol Metab, 2005, 90(8): 4955-4962.
5.	Findling JW, Raff H. Cushing’s Syndrome: important issues in diagnosis and management. J Clin Endocrinol Metab, 2006, 91(10): 3746-3753.
6.	Lacroix A, Feelders RA, Stratakis CA, et al. Cushing’s syndrome. Lancet, 2015, 386(9996): 913-927.
7.	Alexandraki KI, Grossman AB. The ectopic ACTH syndrome. Rev Endocr Metab Disord, 2010, 11(2): 117-126.
8.	Ejaz S, Vassilopoulou-Sellin R, Busaidy NL, et al. Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: the University of Texas MD Anderson Cancer Center Experience. Cancer, 2011, 117(19): 4381-4389.
9.	Liddle GW, Nicholson WE, Island DP, et al. Clinical and laboratory studies of ectopic humoral syndromes. Recent Prog Horm Res, 1969, 25: 283-314.
10.	张小英, 李红. 库欣综合征与低血钾. 中国实用内科杂志, 2001, 21(9): 518-519.
11.	Aniszewski JP, Young WF Jr, Thompson GB, et al. Cushing syndrome due to ectopic adrenocorticotropic hormone secretion. World J Surg, 2001, 25(7): 934-940.
12.	Beuschlein F, Hammer GD. Ectopic pro-opiomelanocortin syndrome. Endocrinol Metab Clin North Am, 2002, 31(1): 191-234.
13.	Isidori AM, Lenzi A. Ectopic ACTH syndrome. Arq Bras Endocrinol Metabol, 2007, 51(8): 1217-1225.
14.	卢琳, 顾锋, 张晓波, 等. 岩下窦静脉取血在疑难 ACTH 依赖性库欣综合征诊断中的应用. 中华神经外科杂志, 2009, 25(12): 1110-1113.
15.	李力, 陈野野, 李单青, 等. 肺部病变所致异位促肾上腺皮质激素综合征的临床诊治经验. 中国肿瘤临床与康复, 2014, 21(12): 1450-1454.
16.	朱燕, 卢琳, 李乃适, 等. 支气管肺类癌导致的异位 ACTH 综合征. 癌症进展, 2010, 8(4): 317-321.
17.	Phan AT, Oberg K, Choi J, et al. NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the thorax (includes lung and thymus). Pancreas, 2010, 39(6): 784-798.
18.	Salgado LR, Fragoso MC, Knoepfelmacher M, et al. Ectopic ACTH syndrome: our experience with 25 cases. Eur J Endocrinol, 2006, 155(5): 725-733.
19.	Öberg K, Hellman P, Ferolla P, et al. Neuroendocrine bronchial and thymic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol, 2012, 23(Suppl 7): v120-v123.
20.	Noel-Savina E, Descourt R. Focus on treatment of lung carcinoid tumor. Onco Targets Ther, 2013, 6: 1533-1537.
21.	Yao JC, Hassan M, Phan A, et al. One hundred years after " carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35, 825 cases in the United States. J Clin Oncol, 2008, 26(18): 3063-3072.
22.	Ettinger DS, Wood DE, Akerley W, et al. NCCN guidelines insights: non-small cell lung cancer, version 4.2016. J Natl Compr Canc Netw, 2016, 14(3): 255-264.
23.	Caplin ME, Baudin E, Ferolla P, et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. Ann Oncol, 2015, 26(8): 1604-1620.
24.	王学斌, 李单青. 晚期肺类癌的诊治进展. 中国胸心血管外科临床杂志, 2017, 24(7): 551-557.

1. Brown HP. Surgical intervention in extra-uterine pregnancy: observations in the treatment of 109 cases at the pennsylvania hospital. Ann Surg, 1928, 87(4): 581-586.
2. Bagshawe KD. Hypokalaemia, carcinoma and Cushing’s syndrome. Lancet, 1960, 2(7145): 284-287.
3. Allott EN, Skelton MO. Increased adrenocortical activity associated with malignant disease. Lancet, 1960, 2(7145): 278-284.
4. Ilias I, Torpy DJ, Pacak K, et al. Cushing’s syndrome due to ectopic corticotropin secretion: twenty years’ experience at the National Institutes of Health. J Clin Endocrinol Metab, 2005, 90(8): 4955-4962.
5. Findling JW, Raff H. Cushing’s Syndrome: important issues in diagnosis and management. J Clin Endocrinol Metab, 2006, 91(10): 3746-3753.
6. Lacroix A, Feelders RA, Stratakis CA, et al. Cushing’s syndrome. Lancet, 2015, 386(9996): 913-927.
7. Alexandraki KI, Grossman AB. The ectopic ACTH syndrome. Rev Endocr Metab Disord, 2010, 11(2): 117-126.
8. Ejaz S, Vassilopoulou-Sellin R, Busaidy NL, et al. Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: the University of Texas MD Anderson Cancer Center Experience. Cancer, 2011, 117(19): 4381-4389.
9. Liddle GW, Nicholson WE, Island DP, et al. Clinical and laboratory studies of ectopic humoral syndromes. Recent Prog Horm Res, 1969, 25: 283-314.
10. 张小英, 李红. 库欣综合征与低血钾. 中国实用内科杂志, 2001, 21(9): 518-519.
11. Aniszewski JP, Young WF Jr, Thompson GB, et al. Cushing syndrome due to ectopic adrenocorticotropic hormone secretion. World J Surg, 2001, 25(7): 934-940.
12. Beuschlein F, Hammer GD. Ectopic pro-opiomelanocortin syndrome. Endocrinol Metab Clin North Am, 2002, 31(1): 191-234.
13. Isidori AM, Lenzi A. Ectopic ACTH syndrome. Arq Bras Endocrinol Metabol, 2007, 51(8): 1217-1225.
14. 卢琳, 顾锋, 张晓波, 等. 岩下窦静脉取血在疑难 ACTH 依赖性库欣综合征诊断中的应用. 中华神经外科杂志, 2009, 25(12): 1110-1113.
15. 李力, 陈野野, 李单青, 等. 肺部病变所致异位促肾上腺皮质激素综合征的临床诊治经验. 中国肿瘤临床与康复, 2014, 21(12): 1450-1454.
16. 朱燕, 卢琳, 李乃适, 等. 支气管肺类癌导致的异位 ACTH 综合征. 癌症进展, 2010, 8(4): 317-321.
17. Phan AT, Oberg K, Choi J, et al. NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the thorax (includes lung and thymus). Pancreas, 2010, 39(6): 784-798.
18. Salgado LR, Fragoso MC, Knoepfelmacher M, et al. Ectopic ACTH syndrome: our experience with 25 cases. Eur J Endocrinol, 2006, 155(5): 725-733.
19. Öberg K, Hellman P, Ferolla P, et al. Neuroendocrine bronchial and thymic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol, 2012, 23(Suppl 7): v120-v123.
20. Noel-Savina E, Descourt R. Focus on treatment of lung carcinoid tumor. Onco Targets Ther, 2013, 6: 1533-1537.
21. Yao JC, Hassan M, Phan A, et al. One hundred years after " carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35, 825 cases in the United States. J Clin Oncol, 2008, 26(18): 3063-3072.
22. Ettinger DS, Wood DE, Akerley W, et al. NCCN guidelines insights: non-small cell lung cancer, version 4.2016. J Natl Compr Canc Netw, 2016, 14(3): 255-264.
23. Caplin ME, Baudin E, Ferolla P, et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. Ann Oncol, 2015, 26(8): 1604-1620.
24. 王学斌, 李单青. 晚期肺类癌的诊治进展. 中国胸心血管外科临床杂志, 2017, 24(7): 551-557.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Clinical retrospective study of 20 patients with ectopic adrenocorticotropic hormone syndrome caused by bronchopulmonary carcinoid

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