From MD Anderson: Cushing Syndrome due to ectopic ACTH

http://www.ncbi.nlm.nih.gov/pubmed/21412758

Cancer. 2011 Mar 15. doi: 10.1002/cncr.26029. [Epub ahead of print]
Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: The University of Texas MD Anderson Cancer Center Experience.
Ejaz S, Vassilopoulou-Sellin R, Busaidy NL, Hu MI, Waguespack SG, Jimenez C, Ying AK, Cabanillas M, Abbara M, Habra MA.

Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas.
Abstract
BACKGROUND: Cushing syndrome (CS) secondary to ectopic adrenocorticotropic hormone (ACTH) secretion (EAS) has been described in association with a variety of tumors. The current experience with this syndrome was based on a few case series and individual case reports. Limited data were available about the tumors associated with CS-EAS in a cancer center setting. In this report, the authors have described their experience with CS-EAS at The University of Texas MD Anderson Cancer Center to further enhance the current understanding and management of this syndrome.

METHODS: This was a retrospective review of 43 patients with CS-EAS who were diagnosed between 1979 and 2009 at The University of Texas MD Anderson Cancer Center.

RESULTS: Different neuroendocrine tumors were associated with CS-EAS. Twenty-one patients (48.9%) had tumors located in the chest cavity, with bronchial carcinoid and small cell lung cancer representing the 2 most common causes. The ACTH source remained occult in 4 patients (9.3%) despite extensive workup. Clinical presentation varied, and the classic features of CS were not evident in some patients. Death occurred in 27 patients (62.8%), and the median overall survival was 32.2 months. Major morbidities included new-onset or worsening hyperglycemia (77%), symptomatic venous thromboembolism (14%), and infections (23%).

CONCLUSIONS: In patients with CS-EAS who attended a comprehensive cancer center, tumors originating in the chest cavity were the leading tumors associated with this syndrome. The authors suspect that CS-EAS is under reported because of the atypical presentation in some patients. Thus, they suggest careful evaluation of patients with neuroendocrine tumors to avoid missing coexisting CS-EAS. Cancer 2011;. © 2011 American Cancer Society.