ACTH-producing neuroendocrine tumor of thymus with recurrences. Clinical case

Cover Page


One of the most difficult in diagnostic and treatment options for endogenous Cushing is the ectopic ACTH syndrome, which causes the development of tumors of different histogenesis localization producing adrenocorticotropic hormone (ACTH), and much less - corticotropin hormone (CRH). ACTH-secreting tumors varied in location, morphological structure and the degree of malignancy. Most of these tumors are characterized by an aggressive course with a propensity to metastasize and relapse. The article presents data of the prevalence, pathogenesis of ectopic ACTH tumors localized in the thymus, analyzis of clinical, morphological features, the methods of diagnosis and treatment. Based on the current literature, the world and our own experience on the diagnosis and treatment of patients with ectopic ACTH syndrome with localization of hormone production in the thymus, we want to highlight the current state of the problem in order to create the most efficient algorithm for diagnostic search and treatment of this difficult group of patients.

About the authors

E A Dobreva

Endocrinology Research Centre, Moscow, Russian Federation


V V Krylov

Endocrinology Research Centre, Moscow, Russian Federation

E I Marova

Endocrinology Research Centre, Moscow, Russian Federation

N S Kuznetsov

Endocrinology Research Centre, Moscow, Russian Federation

N V Latkina

Endocrinology Research Centre, Moscow, Russian Federation


  1. Doi M, Sugiyama T, Izumiyama H, et al. Clinical features and management of ectopic ACTH syndrome at a single institute in Japan. Endocrine J. 2010;57(12):1061-1069. doi: 10.1507/endocrj.K10E-265.
  2. Кузнецов Н.С., Марова Е.И., Латкина Н.В., и др. Трудности в диагностике АКТГ-эктопических опухолей. Клинический случай. // Эндокринная хирургия. - 2014. - Т.8. - №2 - С.9-13. [Kuznetsov NS, Marova EI, Latkina NV, et al. Difficulties in the diagnosis of ACTH-ectopic tumors. Case report. Endocrine Surg. 2014;8(2):9-13. (In Russ).] doi: 10.14341/serg201429-13.
  3. Isidori AM, Lenzi A. Ectopic ACTH syndrome. Arquivos Brasileiros de Endocrinologia & Metabologia. 2007; 51(8):1217-1225. doi: 10.1590/s0004-27302007000800007.
  4. Кузнецов Н.С., Марова Е.И., Ремизов О.В., и др. АКТГ-продуцирующая опухоль слепой кишки. Клинический случай. // Эндокринная хирургия. - 2013. - Т.7. - №2 - С.39-44. [Kuznetsov NS, Marova EI, Remizov OV, et al. Acth-secreting tumor of cecum. Case report. Endocrine Surgery. 2013;(2):39-44. (In Russ).] doi: 10.14341/serg2013239-44.
  5. Кузнецов Н.С., Марова Е.И., Ремизов О.В., и др. АКТГ-продуцирующая опухоль редкой локализации. // Проблемы эндокринологии. - 2014. - Т.60. - №1 - С.46-50. [Kuznetsov NS, Marova EI, Remizov OV, et al. Rare localization of an ACTH-producing tumour. Problemy Endokrinologii. 2014;60(1):46-50. (In Russ).] doi: 10.14341/probl201460146-50.
  6. Rosai J, Higa E. Mediastinal endocrine neoplasm, of probable thymic origin, related to carcinoid tumor. Clinicopathologic study of 8 cases. Cancer. 1972;29(4):1061-1074. doi: 10.1002/1097-0142(197204)29:4<1061:: aid-cncr2820290456>;2-3.
  7. Neary NM, Lopez-Chavez A, Abel BS, et al. Neuroendocrine acth-producing tumor of the thymus-experience with 12 patients over 25 years. J Clin Endocrinol Metab. 2012;97(7):2223-2230. doi: 10.1210/jc.2011-3355
  8. Pass HI, Doppman JL, Nieman L, et al. Management of the ectopic acth syndrome due to thoracic carcinoids. Ann Thorac Surg. 1990;50(1):52-57. doi: 10.1016/0003-4975(90)90085-k.
  9. Moran CA, Suster S. Neuroendocrine carcinomas (carcinoid tumor) of the thymus. A clinicopathologic analysis of 80 cases. Am J Clin Pathol. 2000;114(1):100-110.
  10. Wang DY, Chang DB, Kuo SH, et al. Carcinoid tumours of the thymus. Thorax. 1994;49(4):357-360. doi: 10.1136/thx.49.4.357.
  11. Teh DC. Thymic carcinoids in multiple endocrine neoplasia type 1. J Intern Med. 1998;243(6):501-504. doi: 10.1046/j.1365-2796.1998.00329.x.



Abstract - 2077

PDF (Russian) - 789



Copyright (c) 2015 Добрева Е.А., Крылов В.В., Марова Е.И., Кузнецов Н.С., Латкина Н.В.

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies