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Updated: Sep 13, 2025

Identification of OTX1 and OTX2 As Two Possible Molecular Markers for Sinonasal Carcinomas and Olfactory Neuroblastomas
Published on: February 28, 2019
Ectopic ACTH Secretion Induced by an Olfactory Neuroblastoma: A Case Report
Jérôme Houdu1, Roger Jankowski1, Duc-Trung Nguyen1
1Department of Otolaryngology and Cervico-Facial Surgery, Centre Hospitalier Régional Universitaire de Nancy, Vandoeuvre-Les-Nancy, France.
Abstract:
Background: 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. Methods: 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. Results: The patient was cured of Cushing's syndrome and remained in remission at 10 years of follow-up. Conclusion: 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.
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