title: A rare case of ectopic adrenocorticotropic hormone syndrome in small cell carcinoma of the vagina: a case report.
abstract: OBJECTIVE
  To describe ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) to increase awareness of this condition when treating patients with gynecological small cell carcinoma (SmCC).
  
  
  METHODS
  This is a review of a 61-year-old woman with primary vaginal SmCC and an atypical presentation of Cushing syndrome. This case describes the molecular rationale, caveats in clinical presentation, pathological diagnosis, and management options for patients with this rare syndrome.
  
  
  RESULTS
  After treatment with primary chemotherapy, the patient presented acutely with delirium and metabolic disturbances. This was associated with elevated ACTH and gastrin levels without any tumor staining for ACTH. The patient was initially managed with ketoconazole to control the EAS. Complications developed related to her Cushing syndrome, and she succumbed to complications of her disease.
  
  
  CONCLUSIONS
  Vaginal SmCC complicated by EAS is a rare paraneoplastic syndrome, and this case history outlines the management options for patients with this condition and reviews the pertinent literature.
authors: Division of Gynaecologic Oncology, The Ottawa Hospital, Canada. jweberpals@ottawahospital.on.ca
authors: Johanne
authors: J
authors: Weberpals
authors: Bojana
authors: B
authors: Djordjevic
authors: Mahmoud
authors: M
authors: Khalifa
authors: Amit
authors: A
authors: Oza
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000175:diagnosis*; D001706:Biopsy; D018288:Carcinoma, Small Cell / Q000175:diagnosis / Q000378:metabolism*; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D035583:Rare Diseases / Q000175:diagnosis*; D014057:Tomography, X-Ray Computed; D014621:Vagina / Q000473:pathology; D014625:Vaginal Neoplasms / Q000175:diagnosis / Q000378:metabolism*