title: Bilateral adrenalectomy: lifesaving procedure in severe Cushing syndrome.
abstract: OBJECTIVE
  To discuss the role of bilateral adrenalectomy in Cushing syndrome, as illustrated in a case of severe hypercortisolism that was unresponsive to combination agent medical therapy.
  
  
  METHODS
  We report the clinical, laboratory, imaging, and pathologic findings in a patient with ectopic Cushing syndrome attributable to an adrenocorticotropic hormone (ACTH)-secreting neuroblastoma. In addition, we provide a literature review regarding olfactory neuroblastoma and discuss current and emerging therapeutic options for Cushing syndrome.
  
  
  RESULTS
  A 59-year-old man presented with nasal congestion and neck swelling and was noted to have hypokalemia, hypertension, and hyperglycemia. A nasal biopsy demonstrated a poorly differentiated carcinoma with neuroendocrine features. He was subsequently diagnosed as having ACTH-dependent Cushing syndrome, but despite high-dose combination medical therapy, his condition rapidly deteriorated. Urgent bilateral adrenalectomy provided rapid control of the hypercortisolism, and the patient was later able to undergo an uncomplicated total macroscopic resection of his locally metastatic primary tumor.
  
  
  CONCLUSIONS
  This report describes the challenges in the diagnosis and management of ACTH-dependent Cushing syndrome and highlights the important role that bilateral adrenalectomy can still have in patients with severe hypercortisolism causing life-threatening complications.
authors.affiliation: Department of Medicine, David Geffen School of Medicine at the University of California Los Angeles, Los Angeles, California 90095, USA.
authors.forename: Jennifer Y
authors.initials: JY
authors.lastname: Han
authors.forename: Leili
authors.initials: L
authors.lastname: Mirsadraei
authors.forename: Michael W
authors.initials: MW
authors.lastname: Yeh
authors.forename: Jeffrey D
authors.initials: JD
authors.lastname: Suh
authors.forename: William H
authors.initials: WH
authors.lastname: Yong
authors.forename: Marvin
authors.initials: M
authors.lastname: Bergsneider
authors.forename: Anthony P
authors.initials: AP
authors.lastname: Heaney
mesh_terms: D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000097:blood / Q000378:metabolism; D059186:Chemoradiotherapy, Adjuvant; D016638:Critical Illness; D003480:Cushing Syndrome / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology / Q000601:surgery*; D003937:Diagnosis, Differential; D018304:Esthesioneuroblastoma, Olfactory / Q000175:diagnosis / Q000503:physiopathology* / Q000601:surgery / Q000628:therapy; D006801:Humans; D006854:Hydrocortisone / Q000097:blood / Q000652:urine; D008297:Male; D008875:Middle Aged; D009669:Nose Neoplasms / Q000175:diagnosis / Q000503:physiopathology* / Q000601:surgery / Q000628:therapy; D016879:Salvage Therapy; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone