An exceptionally rare case of Cushing’s syndrome caused by ectopic ACTH syndrome due to olfactory neuroblastoma in childhood

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

doi:10.1515/jpem-2022-0506

Article

An exceptionally rare case of Cushing’s syndrome caused by ectopic ACTH syndrome due to olfactory neuroblastoma in childhood

Bayram Özhan , Didem Yıldırım Çakar , Gülsün Gülten and Nagihan Yalçın

Published/Copyright: January 12, 2023

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From the journal Journal of Pediatric Endocrinology and Metabolism Volume 36 Issue 5

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 long as the treatment was maintained.

Conclusions

Cushing syndrome is a rare endocrine disorder. Adrenal sources of hypercortisolism and ectopic sources of ACTH overproduction should be investigated especially in young children.

Keywords: Cushing’s syndrome; esthesioneuroblastoma; paraneoplastic syndrome

Corresponding author: Bayram Ozhan, MD, Associated Professor, Çocuk Endokrinoloji Polikliniği, Pamukkale Universitesi Tıp Fakültesi Hastanesi, Denizli, Kınıklı, Türkiye, Phone: +905052656283, Fax: +90258 261 92 06, E-mail: bayramozhan@yahoo.com

Research funding: None declared.
Author contributions: All authors have accepted responsibility for the entire content of this manuscript and approved its submission. B.Ö and D.Y. contributed in collecting data and the writing manuscript; G.G and N.Y. contributed in performing pathologic examination and editing the manuscript.
Competing interests: Authors state no conflict of interest.
Informed consent: Written informed consent was obtained from the parents.
Ethical approval: Not applicable.

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Received: 2022-10-12

Accepted: 2022-12-30

Published Online: 2023-01-12

Published in Print: 2023-05-25

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Articles in the same Issue

https://doi.org/10.1515/jpem-2022-0506

Keywords for this article

Cushing’s syndrome; esthesioneuroblastoma; paraneoplastic syndrome