© Georg Thieme Verlag KG Stuttgart · New York
An Overlooked Tumor of the Lung in Cushing's Syndrome: Adrenocorticotropic Hormone-Secreting Carcinoid Tumor
Received November 29, 2006
19 November 2007 (online)
Adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome may result from ectopic ACTH secretion in 15 - 20 % of patients []. Several diagnostic procedures including an endocrine workup and inferior petrosal sinus sampling to exclude a pituitary tumor are usually carried out to establish the origin of ectopic ACTH, however, the source of ectopic ACTH is not easily ascertained []. Moreover, many cases, ranging from 30 - 42 %, unnecessarily undergo either a hypophysectomy or an adrenalectomy [, , ].
We report here a case with Cushing's syndrome resulting from a pulmonary carcinoid tumor diagnosed 13 months following the onset of symptoms. Histological diagnosis revealed a typical carcinoid tumor with mediastinal lymph node involvement. We wish to emphasize this exceptional location of this carcinoid tumor, which may be overlooked unless suspected, and also the necessity of mediastinal lymph node dissection regardless of the nature of the tumor.
- 1 Wajchenberg B L, Mendoca B B, Liberman B. et al . Ectopic adrenocorticotropic hormone syndrome. Endocr Rev. 1995; 15 752-787
- 2 Dall'Astaa C, Santambrogio L, Castellani M, Ambrosi B. Difficulties in diagnosis and treatment of ectopic ACTH-producing tumors of the chest. Eur J Cardiothorac Surg. 2002; 21 149-151
- 3 Deb S J, Nichols F C, Allen M S, Deschamps C, Cassivi S D, Pairolero P C. Pulmonary carcinoid tumors with Cushing's syndrome: an aggressive variant or not?. Ann Thorac Surg. 2005; 79 1132-1136
- 4 Shrager J B, Wright C D, Wain J C, Torchiana D F, Grillo H C, Mathisen D J. Bronchopulmonary carcinoid tumors aasociated with Cushing's syndrome: a more aggressive variant of typical carcinoid. J Thorac Cardiovasc Surg. 1997; 114 367-375
- 5 Dogan R, Oc M, Firat P, Nar A. An ectopic ACTH-producing bronchial carcinoid tumor: report of a case who underwent hypophysectomy unnecessarily. Case Rep Clin Pract Rev. 2003; 4 21-25
- 6 Ozbey N, Bozbora A, Kalayci G. et al . Cushing's syndrome caused by ectopic corticotropin secretion by multiple peripheral pulmonary carcinoids and tumorlets of carcinoid type. J Endocrinol Inves. 2000; 23 536-541
- 7 De Matos L L, Trufelli D C, das Neves-Pereira J C, Danel C, Riquet M. Cushing's syndrome secondary to bronchopulmonary carcinoid tumor: report of two cases and literature review. Lung Cancer. 2006; 53 381-386
- 8 Filosso P L, Rena O, Ruffini E, Oliaro A. Ectopic ACTH-producing tumors of the chest and octreotide scintigraphy. Eur J Cardiothorac Surg. 2002; 21 1126-1129
- 9 Daniels C E, Lowe V J, Aubry M C, Allen M S, Jett J R. The utility of fluorodeoxyglucose positron emission tomography in the evaluation of carcinoid tumors presenting as pulmonary nodules. Chest. 2007; 131 255-260
- 10 Pass H I, Doppman J L, Nieman L. et al . Management of the ectopic ACTH syndrome due to thoracic carcinoids. Ann Thorac Surg. 1990; 50 52-67
- 11 Garcia-Yuste M, Matilla J M, Alvarez-Gago T. et al . The Spanish Multicenter Study of Neuroendocrine Tumors of the Lung of the Spanish Society of Pneumonology and Thoracic Surgery (EMETNE-SEPAR). Prognostic factors in neuroendocrine lung tumors: a Spanish multicenter study. Ann Thorac Surg. 2000; 70 258-263
- 12 Gharagozloo F, Dausmann M J, Whitaker M D. et al . Thoracoscopic excision of a peripheral corticotropin-secreting pulmonary carcinoid tumor. Mayo Clin Proc. 1994; 69 594-596
Murat Kara, Associate Prof.
Department of Thoracic Surgery
Faculty of Medicine
Fax: + 90 31 23 11 17 73