title: Severe ectopic cushing syndrome caused by adenoid cystic carcinoma of a salivary gland.
abstract: OBJECTIVE
  We present a rare case of Cushing syndrome due to ectopic adrenocorticotropic hormone (ACTH) secretion (EAS). To our knowledge only two similar cases have been previously reported.
  
  
  METHODS
  This is a case report of EAS by a metastatic lingual adenoid cystic carcinoma (ACC).
  
  
  RESULTS
  The patient was diagnosed of a Cushing syndrome caused by tumoral EAS two years after initial cancer diagnosis. Clinical presentation included asthenia, insomnia, hypertension, acne, and hyperpigmentation developing in a period of two months. Laboratory and imaging testing revealed hypokalemic metabolic alkalosis, hypercortisolemia, high ACTH, nonsuppresion by 8 mg dexamethasone, and a normal pituitary magnetic resonance imaging (MRI). With a high clinical suspicion of EAS, combined medical treatment was started but was unsuccessful. Bilateral adrenalectomy could not be performed given the patient's rapid deterioration. Immunostained tissue from the original tumor was positive for synaptophysin.
  
  
  CONCLUSIONS
  This rare case of EAS illustrates the challenge that this condition may confer regarding diagnosis and management.
authors.affiliation: Endocrinology Department, EDUAB-HSP Hospital de la Santa Creu I Sant Pau, Barcelona, Spain.
authors.forename: Valeria
authors.initials: V
authors.lastname: Alcantara
authors.forename: Eulalia
authors.initials: E
authors.lastname: Urgell
authors.forename: J Francesc
authors.initials: JF
authors.lastname: Sancho
authors.forename: Ana
authors.initials: A
authors.lastname: Chico
mesh_terms: D000328:Adult; D003528:Carcinoma, Adenoid Cystic / Q000150:complications*; D003480:Cushing Syndrome / Q000175:diagnosis* / Q000209:etiology*; D005260:Female; D006801:Humans; D012469:Salivary Glands / Q000473:pathology*