A Rare Endocrine Manifestation of Esthesioneuroblastoma: A Comprehensive Review of Reported Cases of Cushing's Syndrome Secondary to Olfactory Neuroblastoma

Background/Objectives: Olfactory neuroblastoma (ONB) is a rare malignant tumor of neuroectodermal origin. Rarely, ONB can elicit paraneoplastic manifestations, with Cushing’s syndrome (CS) due to ectopic adrenocorticotropic hormone (ACTH) secretion being extremely uncommon but clinically significant. Methods: We performed a literature review of CS and ONB and reviewed 37 reported cases from 34 studies. Results: Patients ranged from infancy to older adulthood, with no gender predominance, and frequently presented with severe hypercortisolism marked by hypokalemia, hypertension, hyperglycemia, and opportunistic infections. Diagnostic challenges include overlap with ACTH-dependent Cushing’s disease and misleading inferior petrosal sinus sampling. Conclusion: Effective management required multidisciplinary collaboration, integrating medical stabilization of cortisol excess with definitive tumor-directed therapy. Surgical resection, often combined with radiotherapy, offered the best chance for long-term remission, though bilateral adrenalectomy was occasionally required in severe cases for urgent control. Despite its rarity, heightened clinical suspicion and coordinated treatment can achieve remission. Future progress will depend on prospective case collection and molecular studies to clarify pathogenesis and guide therapy. Publication History Article published online: 27 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany