Volume 8 Issue 2-3
Oct.  2020
Turn off MathJax
Article Contents
Cheng HUANG, Ye-ye CHEN, Shan-qing LI, Xiao-yun ZHOU, Hong-sheng LIU, Li LI, Ying-zhi QIN, Jia HE, Dong-jie MA. Ectopic Adrenocorticotropic Hormone Syndrome Caused by Thoracic Neuroendocrine Tumors: Surgical Treatment and Prognosis Factors[J]. Medical Journal of Peking Union Medical College Hospital, 2017, 8(2-3): 147-153. doi: 10.3969/j.issn.1674-9081.2017.03.012
Citation: Cheng HUANG, Ye-ye CHEN, Shan-qing LI, Xiao-yun ZHOU, Hong-sheng LIU, Li LI, Ying-zhi QIN, Jia HE, Dong-jie MA. Ectopic Adrenocorticotropic Hormone Syndrome Caused by Thoracic Neuroendocrine Tumors: Surgical Treatment and Prognosis Factors[J]. Medical Journal of Peking Union Medical College Hospital, 2017, 8(2-3): 147-153. doi: 10.3969/j.issn.1674-9081.2017.03.012

Ectopic Adrenocorticotropic Hormone Syndrome Caused by Thoracic Neuroendocrine Tumors: Surgical Treatment and Prognosis Factors

doi: 10.3969/j.issn.1674-9081.2017.03.012
More Information
  • Corresponding author: MA Dong-jie   Tel: 010-69152630, Email:pumchlsq@163.com
  • Received Date: 2016-08-04
  • Publish Date: 2020-10-30
  •     Objective       To discuss the surgical management and prognosis factors of ectopic adrenocorticotropic hormone syndrome (EAS) caused by thoracic neuroendocrine tumors(NETs).    Methods       The clinical manifestations, radiological findings, outcome of surgical treatment and follow-up of consecutive 33 cases(20 males and 13 females) of EAS caused by thoracic NETs from December 1983 to December 2013 in Peking Union Medical College Hospital were retrospectively analyzed.    Results       The median age was 33 years old (13~65 years)and the median duration of disease was 12 months(1~156 months). Cushing's syndrome was demonstrated in all cases by clinical and biochemical evidences. All the thoracic NETs were located by computed tomography (CT) eventually. The serum cortisol and adrenocorticotropic hormone levels decreased significantly after surgery(both P < 0.05).One patient(3.0%) died after surgery and two patients(6.1%) had postoperative complications.The overall 5-year survival rate was 65.8%.Pulmonary diseases had better survival rate versus thymic diseases(84.2% vs. 36.3%, P < 0.05).The 5-year survival rate was 76.2% in typical carcinoid and 45.7% in atypical carcinoid(P=0.056).The 5-year survival rate was 80.0% in stage Ⅰ and Ⅱ, 50.5% in stage Ⅲ and Ⅳ respectively(P=0.072).    Conclusions       Due to the varity and atypical clinical features, early diagnosis and therapy in EAS is very challenging for clinicians. CT scan plays a key role in localization of the thoracic NETs. Pulmonary diseases have better prognosis than thymic diseases.Pathological type and stage of tumors might be prognostic factors for this rare syndrome.
  • loading
  • [1] Han B, Sun JM, Ahn JS, et al.Clinical outcomes of atypical carcinoid tumors of the lung and thymus:7-year experience of a rare malignancy at single institute[J]. Med Oncol, 2013, 30:479. doi:  10.1007/s12032-013-0479-x
    [2] Oberg K, Hellman P, Ferolla P, et al. Neuroendocrine bronchial and thymic tumors:ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up[J]. Ann Oncol, 2012, 23 Suppl 7:120-123. https://academic.oup.com/annonc/article/23/suppl_7/vii120/144890
    [3] 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]. J Clin Oncol, 2008, 26:3063-3072. doi:  10.1200/JCO.2007.15.4377
    [4] 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)[J]. Pancreas, 2010, 39:784-798. doi:  10.1097/MPA.0b013e3181ec1380
    [5] Travis WD, Brambilla E, Müller-Hermelink, et al.Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart (WHO Classification of Tumours)[M]. Lyon, France:IARC Press, 2004:145-247.
    [6] Lacroix A, Feelders RA, Stratakis CA, et al. Cushing's syndrome[J]. Lancet, 2015, 386:913-927. doi:  10.1016/S0140-6736(14)61375-1
    [7] Lababede O, Meziane M, Rice T.Seventh edition of the cancer staging manual and stage grouping of lung cancer:quick reference chart and diagrams[J]. Chest, 2011, 139:183-189. doi:  10.1378/chest.10-1099
    [8] Detterbeck FC, Nicholson AG, Kondo K, et al.The Masaoka-Koga stage classification for thymic malignancies:clarification and definition of terms[J]. J Thorac Oncol, 2011, 6:S1710-S1716. doi:  10.1097/JTO.0b013e31821e8cff
    [9] Alexandraki KI, Grossman AB.The ectopic ACTH syndrome[J]. Rev Endocr Metab Disord, 2010, 11:117-126. doi:  10.1007/s11154-010-9139-z
    [10] Isidori AM, Lenzi A.Ectopic ACTH syndrome[J]. Arq Bras Endocrinol Metabol, 2007, 51:1217-1225. doi:  10.1590/S0004-27302007000800007
    [11] 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[J]. Cancer, 2011, 117:4381-4389. doi:  10.1002/cncr.26029
    [12] Liddle GW, Nicholson WE, Island DP, et al. Clinical and laboratory studies of ectopic humoral syndromes[J]. Recent Prog Horm Res, 1969, 25:283-314. http://www.ncbi.nlm.nih.gov/pubmed/4310919
    [13] Wajchenberg BL, Mendonca B, Liberman B, et al. Ectopic ACTH syndrome[J]. J Steroid Biochem Mol Biol, 1995, 53:139-151. doi:  10.1016/0960-0760(95)00044-Z
    [14] Isidori AM, Kaltsas GA, Pozza C, et al. The ectopic adrenocorticotropin syndrome:clinical features, diagnosis, management, and long-term follow-up[J]. J Clin Endocrinol Metab, 2006, 91:371-377. doi:  10.1210/jc.2005-1542
    [15] Aniszewski JP, Young WF, Thompson GB, et al. Cushing syndrome due to ectopic adrenocorticotropic hormone secretion[J]. World J Surg, 2001, 25:934-940. doi:  10.1007/s00268-001-0032-5
    [16] Beuschlein F, Hammer GD.Ectopic pro-opiomelanocortin syndrome[J]. Endocrinol Metab Clin North Am, 2002, 31:191-234. doi:  10.1016/S0889-8529(01)00025-1
    [17] 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[J].中华神经外科杂志, 2009, 25:1110-1113. doi:  10.3760/cma.j.issn.1001-2346.2009.012.022
    [18] Findling JW, Kehoe ME, Shaker JL, et al. Routine inferior petrosal sinus sampling in the differential diagnosis of adrenocorticotropin (ACTH)-dependent Cushing's syndrome:early recognition of the occult ectopic ACTH syndrome[J]. J Clin Endocrinol Metab, 1991, 73:408-413. doi:  10.1210/jcem-73-2-408
    [19] De Herder WW, Lamberts SW.Octapeptide somatostatin-analogue therapy of Cushing's syndrome[J]. Postgrad Med J, 1999, 75:65-66. doi:  10.1136/pgmj.75.880.65
    [20] Salgado LR, Fragoso MC, Knoepfelmacher M, et al. Ectopic ACTH syndrome:our experience with 25 cases[J]. Eur J Endocrinol, 2006, 155:725-733. doi:  10.1530/eje.1.02278
    [21] Noel-Savina E, Descourt R.Focus on treatment of lung carcinoid tumor[J]. Oncol Targets Ther, 2013, 6:1533-1537. http://www.ncbi.nlm.nih.gov/pubmed/24187503
    [22] Crona J, Bjorklund P, Welin S, et al. Treatment, prognostic markers and survival in thymic neuroendocrine tumours. a study from a single tertiary referral centre[J]. Lung Cancer, 2013, 79:289-293. doi:  10.1016/j.lungcan.2012.12.001
    [23] 陈野野, 刘洪生, 李单青, 等.胸腺神经内分泌肿瘤手术治疗及预后因素[J].协和医学杂志, 2016, 7:190-194. doi:  10.3969/j.issn.1674-9081.2016.03.006
    [24] Neary NM, Lopez-Chavez A, Abel BS, et al. Neuroendocrine ACTH-producing tumor of the thymus-experience with 12 patients over 25 years[J]. J Clin Endocrinol Metab, 2012, 97:2223-2230. doi:  10.1210/jc.2011-3355
    [25] Kyriss T, Maier S, Veit S, et al. Carcinoid lung tumors:long-term results from 111 resections[J]. Thorac Surg Sci, 2006, 3:Doc03. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3011338/
    [26] Gaur P, Leary C, Yao JC.Thymic neuroendocrine tumors:a SEER database analysis of 160 patients[J]. Ann Surg, 2010, 251:1117-1121. doi:  10.1097/SLA.0b013e3181dd4ec4
    [27] Garcia-Yuste M, Matilla JM, Cueto A, et al. Typical and atypical carcinoid tumours:analysis of the experience of the Spanish multi-centric study of neuroendocrine tumours of the lung[J]. Eur J Cardiothorac Surg, 2007, 31:192-197. doi:  10.1016/j.ejcts.2006.11.031
    [28] Moran CA, Suster S.Neuroendocrine carcinomas (carcinoid tumor) of the thymus. A clinicopathologic analysis of 80 cases[J]. Am J Clin Pathol, 2000, 114:100-110. http://med.wanfangdata.com.cn/Paper/Detail/PeriodicalPaper_JJ021061893
    [29] Cardillo G, Rea F, Lucchi M, et al. Primary neuroendocrine tumors of the thymus:a multicenter experience of 35 patients[J]. Ann Thorac Surg, 2012, 94:241-246. doi:  10.1016/j.athoracsur.2012.03.062
    [30] Oberg K. Chemotherapy and biotherapy in the treatment of neuroendocrine tumours[J]. Ann Oncol, 2001, 12 Suppl 2:S111-S114. http://www.ncbi.nlm.nih.gov/pubmed/11762335
  • 加载中


    通讯作者: 陈斌, bchen63@163.com
    • 1. 

      沈阳化工大学材料科学与工程学院 沈阳 110142

    1. 本站搜索
    2. 百度学术搜索
    3. 万方数据库搜索
    4. CNKI搜索

    Figures(3)  / Tables(2)

    Article Metrics

    Article views (236) PDF downloads(2) Cited by()
    Proportional views


    DownLoad:  Full-Size Img  PowerPoint