World Journal of Endocrine Surgery

Register      Login

VOLUME 10 , ISSUE 1 ( January-April, 2018 ) > List of Articles


Adrenocorticotropic Hormone-producing Neuroendocrine Tumors of Thymus: Case Series and Review of Literature

Sushil Gupta, Niraj Kumari, Roma Pradhan

Keywords : Adrenocorticotropic hormone, Cushing's syndrome, Neuroendocrine tumors

Citation Information : Gupta S, Kumari N, Pradhan R. Adrenocorticotropic Hormone-producing Neuroendocrine Tumors of Thymus: Case Series and Review of Literature. World J Endoc Surg 2018; 10 (1):1-4.

DOI: 10.5005/jp-journals-10002-1225_1

License: CC BY-NC 4.0

Published Online: 01-12-2018

Copyright Statement:  Copyright © 2018; The Author(s).


Since the first description of thymic carcinoid as a specific entity in 1972 by Rosai and Higa, approximately 92 cases of adrenocorticotropic hormone (ACTH)-producing thymic neuroendocrine associated with Cushing's syndrome (CS) have been described between 1980 and 2011. We report here three new cases of neuroendocrine tumor (NET) of thymus associated with ectopic production of ACTH along with review of recent literature. All our three cases were middle age (2 male and 1 female) with clinical features of CS. Two of the three patients had hyperpigmentation. All the three underwent transsternal excision of thymic tumor. The NET of the thymus associated with CS can occur at any age from 4 to 64 years; however, meta-analysis revealed that majority of cases occur between 18 and 40 years. There is no sex predilection for these tumors except for the NETs which are associated with MEN-1, which occurred predominantly in males. Majority of these patients would present with clinical features of CS, although the severity and rapidity of onset would vary. The ACTH-producing NET is a rare cause of CS and requires high suspicion to make an early diagnosis and is a locally aggressive disease that requires aggressive surgical resection. Adjuvant radiotherapy may be beneficial.

PDF Share
  1. Rosai J, Higa E. Mediastinal endocrine neoplasm of probable thymic origin, related to carcinoid tumor. Clinicopathologic study of 8 cases. Cancer 1972 Apr;29(4):1061-1074.
  2. Moran CA, Suster S. Neuroendocrine carcinomas (carcinoid tumor) of the thymus. A clinicopathologic analysis of 80 cases. Am J Clin Pathol 2000 Jul;114(1):100-110.
  3. Neary NM, Lopez-Chavez A, Abel BS, Boyce AM, Schaub N, Kwong K, Stratakis CA, Moran CA, Giaccone G, Nieman LK. Neuroendocrine ACTH-producing tumor of the thymus – experience with 12 patients over 25 years. J Clin Endocrinol Metab 2012 Jul;97(7):2223-2230.
  4. Sathyakumar S, Paul TV, Asha HS, Gnanamuthu BR, Paul MJ, Abraham DT, Rajaratnam S, Thomas N. Ectopic Cushing syndrome: a 10-year experience from a tertiary care center in southern India. Endocr Pract 2017 Aug;23(8):907-914.
  5. de Perrot M, Spiliopoulos A, Fischer S, Totsch M, Keshavjee S. Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome. Ann Thorac Surg 2002 Feb;73(2):675-681.
  6. McCaughey ES, Walker V, Rolles CJ, Scheurmier NI, Hale AC, Rees LH. Ectopic ACTH production by a thymic carcinoid tumour. Eur J Pediatr 1987 Nov;146(6):590-591.
  7. Lin KL, Chen CY, Hsu HH, Kao PF, Huang MJ, Wang HS. Ectopic ACTH syndrome due to thymic carcinoid tumor in a girl. J Pediatr Endocrinol Metab 1999 Jul-Aug;12(4):573-578.
  8. Ghazi AA, Dezfooli AA, Mohamadi F, Yousefi SV, Amirbaigloo A, Ghazi S, Pourafkari M, Berney D, Ellard S, Grossman AB. Cushing syndrome secondary to a thymic carcinoid tumor due to multiple endocrine neoplasia type 1. Endocr Pract 2011 Jul-Aug;17(4):e92-e96.
  9. Pass HI, Doppman JL, Nieman L, Stovroff M, Vetto J, Norton JA, Travis W, Chrousos GP, Oldfield EH, Cutler GB Jr. Management of the ectopic ACTH syndrome due to thoracic carcinoids. Ann Thorac Surg 1990 Jul;50(1):52-57.
  10. Klemm KM, Moran CA. Primary neuroendocrine carcinomas of the thymus. Semin Diagn Pathol 1999 Feb;16(1):32-41.
  11. Wick MR. Neuroendocrine neoplasia. Current concepts. Am J Clin Pathol 2000 Mar;113(3):331-335.
  12. Ruffini E, Oliaro A, Novero D, Campisi P, Filosso PL. Neuroendocrine tumors of the thymus. Thorac Surg Clin 2011 Feb;21(1):13-23.
  13. Wick MR, Scott RE, Li CY, Carney JA. Carcinoid tumor of the thymus: a clinicopathologic report of seven cases with a review of the literature. Mayo Clin Proc 1980 Apr;55(4):246-254.
  14. Takagi J, Otake K, Morishita M, Kato H, Nakao N, Yoshikawa K, Ikeda H, Hirooka Y, Hattori Y, Larsson C, et al. Multiple endocrine neoplasia type I and Cushing's syndrome due to an aggressive ACTH producing thymic carcinoid. Intern Med 2006 Feb;45(2):81-86.
PDF Share
PDF Share

© Jaypee Brothers Medical Publishers (P) LTD.