Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose
ACTH-dependent hypercortisolism is caused by an ectopic ACTH syndrome (EAS) in 20% of cases. We report a rare cause of EAS in a 41-year-old woman, presenting with clinical features of Cushing’s syndrome which developed over several months. Biochemical tests revealed hypokalemic metabolic alkalosis a...
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| Format: | Article |
| Language: | English |
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Bioscientifica
2019-10-01
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| Series: | Endocrinology, Diabetes & Metabolism Case Reports |
| Online Access: | https://edm.bioscientifica.com/view/journals/edm/2019/1/EDM19-0093.xml |
| _version_ | 1830297816557158400 |
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| author | Karen Decaestecker Veerle Wijtvliet Peter Coremans Nike Van Doninck |
| author_facet | Karen Decaestecker Veerle Wijtvliet Peter Coremans Nike Van Doninck |
| author_sort | Karen Decaestecker |
| collection | DOAJ |
| description | ACTH-dependent hypercortisolism is caused by an ectopic ACTH syndrome (EAS) in 20% of cases. We report a rare cause of EAS in a 41-year-old woman, presenting with clinical features of Cushing’s syndrome which developed over several months. Biochemical tests revealed hypokalemic metabolic alkalosis and high morning cortisol and ACTH levels. Further testing, including 24-hour urine analysis, late-night saliva and low-dose dexamethasone suppression test, confirmed hypercortisolism. An MRI of the pituitary gland was normal. Inferior petrosal sinus sampling (IPSS) revealed inconsistent results, with a raised basal gradient but no rise after CRH stimulation. Additional PET-CT showed intense metabolic activity in the left nasal vault. Biopsy of this lesion revealed an unsuspected cause of Cushing’s syndrome: an olfactory neuroblastoma (ONB) with positive immunostaining for ACTH. Our patient underwent transnasal resection of the tumour mass, followed by adjuvant radiotherapy. Normalisation of cortisol and ACTH levels was seen immediately after surgery. Hydrocortisone substitution was started to prevent withdrawal symptoms. As the hypothalamic–pituitary–axis slowly recovered, daily hydrocortisone doses were tapered and stopped 4 months after surgery. Clinical Cushing’s stigmata improved gradually. |
| first_indexed | 2024-12-19T07:40:57Z |
| format | Article |
| id | doaj.art-e72fdc8bc01349c095edf81030c92394 |
| institution | Directory Open Access Journal |
| issn | 2052-0573 2052-0573 |
| language | English |
| last_indexed | 2024-12-19T07:40:57Z |
| publishDate | 2019-10-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrinology, Diabetes & Metabolism Case Reports |
| spelling | doaj.art-e72fdc8bc01349c095edf81030c923942022-12-21T20:30:28ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732052-05732019-10-01111610.1530/EDM-19-0093Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your noseKaren Decaestecker0Veerle Wijtvliet1Peter Coremans2Nike Van Doninck3Department of Diabetology-Endocrinology, AZ Nikolaas, Sint-Niklaas, BelgiumDepartment of Diabetology-Endocrinology, AZ Nikolaas, Sint-Niklaas, BelgiumDepartment of Diabetology-Endocrinology, AZ Nikolaas, Sint-Niklaas, BelgiumDepartment of Diabetology-Endocrinology, AZ Nikolaas, Sint-Niklaas, BelgiumACTH-dependent hypercortisolism is caused by an ectopic ACTH syndrome (EAS) in 20% of cases. We report a rare cause of EAS in a 41-year-old woman, presenting with clinical features of Cushing’s syndrome which developed over several months. Biochemical tests revealed hypokalemic metabolic alkalosis and high morning cortisol and ACTH levels. Further testing, including 24-hour urine analysis, late-night saliva and low-dose dexamethasone suppression test, confirmed hypercortisolism. An MRI of the pituitary gland was normal. Inferior petrosal sinus sampling (IPSS) revealed inconsistent results, with a raised basal gradient but no rise after CRH stimulation. Additional PET-CT showed intense metabolic activity in the left nasal vault. Biopsy of this lesion revealed an unsuspected cause of Cushing’s syndrome: an olfactory neuroblastoma (ONB) with positive immunostaining for ACTH. Our patient underwent transnasal resection of the tumour mass, followed by adjuvant radiotherapy. Normalisation of cortisol and ACTH levels was seen immediately after surgery. Hydrocortisone substitution was started to prevent withdrawal symptoms. As the hypothalamic–pituitary–axis slowly recovered, daily hydrocortisone doses were tapered and stopped 4 months after surgery. Clinical Cushing’s stigmata improved gradually.https://edm.bioscientifica.com/view/journals/edm/2019/1/EDM19-0093.xml |
| spellingShingle | Karen Decaestecker Veerle Wijtvliet Peter Coremans Nike Van Doninck Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose Endocrinology, Diabetes & Metabolism Case Reports |
| title | Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose |
| title_full | Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose |
| title_fullStr | Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose |
| title_full_unstemmed | Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose |
| title_short | Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose |
| title_sort | olfactory neuroblastoma esthesioneuroblastoma presenting as ectopic acth syndrome always follow your nose |
| url | https://edm.bioscientifica.com/view/journals/edm/2019/1/EDM19-0093.xml |
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