Ectopic Cushing’s syndrome secondary to olfactory neuroblastoma

• Published in: Acta neurochirurgica Vol. 160; no. 5; pp. 1023 - 1026
• Main Authors: Yu, Kenny, Roncaroli, Federico, Kearney, Tara, Ewins, David, Beeharry, Deepa, Naylor, Thomas, Ray, David, Bhalla, Rajiv, Gnanalingham, Kanna
• Format: Journal Article
• Language: English
• Published: Vienna Springer Vienna 01.05.2018; Springer Nature B.V
• Subjects: Adrenal glands; Adrenocorticotropic hormone; Case Report - Brain Tumors; Cortisol; Cushing syndrome; Cushing Syndrome - etiology; Cushing Syndrome - pathology; Esthesioneuroblastoma, Olfactory - complications; Esthesioneuroblastoma, Olfactory - pathology; Humans; Interventional Radiology; Male; Medicine; Medicine & Public Health; Middle Aged; Minimally Invasive Surgery; Nervous system diseases; Neuroblastoma; Neurology; Neuroradiology; Neurosurgery; Nose Neoplasms - complications; Nose Neoplasms - pathology; Pituitary; Secretion; Surgery; Surgical Orthopedics; ACTH; Cushing’s; Ectopic; Olfactory neuroblastoma
• ISSN: 0001-6268; 0942-0940
• DOI: 10.1007/s00701-017-3447-y

We present the case of a patient with Cushing’s syndrome secondary to ectopic ACTH secretion. A MR of the head showed a left-sided nasal mass extending down from the cribriform plate. The patient underwent endoscopic resection with nearly complete removal of the mass. Histological examination showed an ACTH-secreting olfactory neuroblastoma (ONB). The patient’s cortisol levels returned to normal range after surgery and have remained normal for over a year. ONB is a rare cause for ectopic ACTH secretion. This case highlights the diagnostic and management difficulties in patients with ectopic ACTH secretion, and provides a brief review of ONB.