Ectopic ACTH Secretion Induced by an Olfactory Neuroblastoma: A Case Report.

Fiche publication

Date publication

juillet 2025

Journal

Case reports in endocrinology

Auteurs

Membres identifiés du Cancéropôle Est :
Pr JANKOWSKI Roger

Tous les auteurs :
Houdu J, Jankowski R, Nguyen DT

Lien Pubmed

https://www.ncbi.nlm.nih.gov/pubmed/40718625

Résumé

Olfactory neuroblastoma (ONB) is a rare tumor of the nasal cavity. It may sometimes present with Cushing's syndrome due to adrenocorticotropic hormone (ACTH) secretion, making it challenging to diagnose. A 65-year-old man with hypokalemia and general weakness presented to the emergency department for Cushing's syndrome. Brain imaging revealed a tumor originating from the ethmoid bone with peritumoral cysts. The first biopsy suggested an ectopic corticotropic pituitary adenoma or a well-differentiated neuroendocrine tumor. However, the second biopsy confirmed an ONB, as suspected by the otolaryngologist. Treatment consisted of neoadjuvant chemotherapy, surgery, and radiotherapy. The patient was cured of Cushing's syndrome and remained in remission at 10 years of follow-up. An unusual mode of discovering ONB is via the diagnosis of Cushing's syndrome caused by ACTH secretion, which may manifest throughout the course of follow-up. Imaging analysis and discussion with pathologists are essential to achieve an accurate diagnosis.