A rare case of ectopic ACTH syndrome due to recurrence of olfactory neuroblastoma

Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a condition of endogenous hypercortisolism sustained by an extrapituitary ACTH-secreting tumor. Olfactory neuroblastoma (ONB) is a rare malignant neoplasm of the sinonasal tract and is derived from the olfactory epithelium. Because the paranasal sinus is not a common site of EAS, the development of ONB in patients with EAS is rare. We herein report the first known case of ONB with acquirement of ACTH production during the clinical course as proven by immunohistochemistry. A 50-year-old man diagnosed with ONB was referred to our department in July 2015 because of hypokalemia, hyperglycemia, decreased eosinophil and granulocyte counts, and elevated serum levels of ACTH and cortisol. Although two previous ONB biopsy specimens (2011 and 2014) showed no ACTH immunoreactivity, a newly obtained specimen in August 2015 clearly showed ACTH immunoreactivity. This is the first case of ectopic ACTH syndrome associated with an ONB that acquired the ability to express ACTH during its clinical course as shown by serial immunohistochemical examinations.

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A rare case of ectopic ACTH syndrome with rhabdomyolysis

BMC Endocrine Disorders ◽

10.1186/s12902-021-00755-0 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Wei Qiang ◽

Sucai Song ◽

Tianjun Chen ◽

Zhe Wang ◽

Jun Feng ◽

...

Keyword(s):

Rare Case ◽

Rare Complication ◽

Clinical Manifestations ◽

Dexamethasone Suppression Test ◽

Ectopic Acth Syndrome ◽

High Dose ◽

Ectopic Acth ◽

Supraclavicular Fossa ◽

Suppression Test ◽

Dexamethasone Suppression

Abstract Background Manifestations of hypokalaemia in ectopic adrenocorticotropic hormonesyndrome(EAS) vary from mild muscle weakness to life-threatening arrhythmia. Herein, we present a rare case of EAS with concomitant rhabdomyolysis(RM) as a result of intractable hypokalaemia. Case presentation A 64-year-old man was admitted for limb weakness and facial hyperpigmentation for 2 weeks. Lab tests revealed intractable hypokalaemia (lowest at 1.8 mmol/L) and metabolic alkalosis. The diagnosis of RM was based on a creatine kinase(CK)level of 5 times the upper limit. The elevated CK and myohemoglobin (Mb) levels returned to within the normal range after the alleviation of hypokalaemia. The patient was diagnosed with ACTH-dependent Cushing’s syndrome (CS) based on unsuppressed serum cortisol after a low-dose dexamethasone suppression test(LDDST) and remarkably elevated ACTH levels. The diagnosis of EAS was made based on the results of a high-dose dexamethasone suppression test(HDDST) and bilateral inferior petrosal sinus sampling(BIPSS). Multiple lymph nodes in the left supraclavicular fossa, right root of neck, mediastinum and bilateral hili of the lung were found with abnormal uptake of 68Ga-DOTA-NOC. Mediastinoscopic lymph node biopsy was performed. The pathological diagnosis was small-cell and large-cell neuroendocrine carcinoma with positive ACTH staining. The patient was prescribed mifepristone and received one cycle of chemotherapy. The patient could not tolerate subsequent chemotherapy and died of dyscrasia. Conclusions RM is a rare complication of EAS with insidious onset and atypical clinical manifestations. Serum potassium levels should be vigilantly monitored to avoid RM in EAS.

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Abstract #1086883: A Rare Case of Ectopic ACTH Syndrome Associated With a Hepatocellular Carcinoma

Endocrine Practice ◽

10.1016/j.eprac.2021.08.027 ◽

2021 ◽

Vol 27 (12) ◽

pp. S8-S9

Author(s):

Ilja Hulinsky ◽

Abraham Rodriguez ◽

Armando Vera

Keyword(s):

Hepatocellular Carcinoma ◽

Rare Case ◽

Ectopic Acth Syndrome ◽

Ectopic Acth

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Refractory Post-Herpetic Neuralgia As An Initial Presentation Of Olfactory Neuroblastoma-Related Ectopic ACTH Syndrome

International Journal of Gerontology ◽

10.1016/s1873-9598(09)70024-9 ◽

2009 ◽

Vol 3 (1) ◽

pp. 81-84 ◽

Cited By ~ 1

Author(s):

Hsiang-Hung Lin ◽

Chih-Ming Lin ◽

Hsiang-Kuang Tseng

Keyword(s):

Olfactory Neuroblastoma ◽

Initial Presentation ◽

Ectopic Acth Syndrome ◽

Post Herpetic Neuralgia ◽

Ectopic Acth

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Olfactory neuroblastoma (esthesioneuroblastoma) presenting as ectopic ACTH syndrome: always follow your nose

Endocrinology Diabetes and Metabolism Case Reports ◽

10.1530/edm-19-0093 ◽

2019 ◽

Vol 2019 ◽

Author(s):

Karen Decaestecker ◽

Veerle Wijtvliet ◽

Peter Coremans ◽

Nike Van Doninck

Keyword(s):

Pituitary Gland ◽

Cushing’S Syndrome ◽

Olfactory Neuroblastoma ◽

Cushing's Syndrome ◽

Ectopic Acth Syndrome ◽

Tumour Mass ◽

List Type ◽

Ectopic Acth ◽

Ectopic Acth Secretion ◽

Late Night

Summary 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. Learning points: Ectopic ACTH syndrome can originate from tumours outside the thoracoabdominal region, like the sinonasal cavity. The diagnostic accuracy of IPSS is not 100%: both false positives and false negatives may occur and might be due to a sinonasal tumour with ectopic ACTH secretion. Olfactory neuroblastoma (syn. esthesioneuroblastoma), named because of its sensory (olfactory) and neuroectodermal origin in the upper nasal cavity, is a rare malignant neoplasm. It should not be confused with neuroblastoma, a tumour of the sympathetic nervous system typically occurring in children. If one criticises MRI of the pituitary gland because of ACTH-dependent hypercortisolism, one should take a close look at the sinonasal field as well.

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