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An exceptionally rare case of Cushing’s syndrome caused by ectopic ACTH syndrome due to olfactory neuroblastoma in childhood by Bayram Özhan; Didem Yıldırım Çakar; Gülsün Gülten; Nagihan Yalçın is a Medicine article available to read on EtoBox.

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## Abstract ## Objectives Ectopic adrenocorticotropic hormone secretion/syndrome (EAS) is caused by excess secretion of ACTH leading to hypercortisolism by non-pituitary, commonly malignant origins. We present a rare case of esthesioneuroblastoma (ENB) complicated by EAS in the follow-up period. ## Case presentation A child presented with nasal obstruction at the age of 10 months. Polypoid mass obstructing the right nasal passage was detected. Magnetic resonance imaging (MRI) showed a lesion limited within the nasal cavity. The lesion was completely removed by nasal endoscopic surgery. The pathologic examination revealed a diagnosis of esthesioneuroblastoma. It was confined to the nasal cavity so chemotherapy/radiotherapy was not administered and began to follow up. At 28 months of age, he presented with rapid weight gain. Laboratory data were consistent with Cushing’s syndrome (CS). High-dose dexamethasone suppression test and imaging studies led us to think of ectopic ACTH syndrome originated from ENB relapse. After partial resection of the tumor, ketoconazole treatment was started along with chemotherapy. Hypercortisolemia was kept under control with ketoconazole treatment as lo

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Author

Bayram Özhan; Didem Yıldırım Çakar; Gülsün Gülten; Nagihan Yalçın

Publisher

Walter de Gruyter GmbH

Published

2023

Language

EN

Field

Medicine (Health Sciences)