title: Cushing syndrome secondary to ectopic adrenocorticotropic hormone secretion: the University of Texas MD Anderson Cancer Center Experience.
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.
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
authors.forename: Shamim
authors.initials: S
authors.lastname: Ejaz
authors.forename: Rena
authors.initials: R
authors.lastname: Vassilopoulou-Sellin
authors.forename: Naifa L
authors.initials: NL
authors.lastname: Busaidy
authors.forename: Mimi I
authors.initials: MI
authors.lastname: Hu
authors.forename: Steven G
authors.initials: SG
authors.lastname: Waguespack
authors.forename: Camilo
authors.initials: C
authors.lastname: Jimenez
authors.forename: Anita K
authors.initials: AK
authors.lastname: Ying
authors.forename: Maria
authors.initials: M
authors.lastname: Cabanillas
authors.forename: Maher
authors.initials: M
authors.lastname: Abbara
authors.forename: Mouhammed Amir
authors.initials: MA
authors.lastname: Habra
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D000368:Aged; D002283:Carcinoma, Bronchogenic / Q000175:diagnosis / Q000209:etiology / Q000378:metabolism; D015897:Comorbidity; D003480:Cushing Syndrome / Q000150:complications*; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D011379:Prognosis; D012189:Retrospective Studies; D055752:Small Cell Lung Carcinoma / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism; D015996:Survival Rate; D055815:Young Adult
chemical_list: D000324:Adrenocorticotropic Hormone