scholarly journals Esthesioneuroblastoma (Olfactory Neuroblastoma) with Ectopic ACTH Syndrome: A Multidisciplinary Case Presentation from the Joan Karnell Cancer Center of Pennsylvania Hospital

2010 ◽  
Vol 15 (1) ◽  
pp. 51-58 ◽  
Author(s):  
David M. Mintzer ◽  
Sarah Zheng ◽  
Michiko Nagamine ◽  
Jason Newman ◽  
Maria Benito
Keyword(s):  
Olfactory Neuroblastoma ◽  
Ectopic Acth Syndrome ◽  
Cancer Center ◽  
Ectopic Acth ◽  
Case Presentation ◽  
Pennsylvania Hospital

scholarly journals Olfactory Neuroblastoma Causing Ectopic ACTH Syndrome

2005 ◽  
Vol 52 (6) ◽  
pp. 675-681 ◽  
Author(s):  
Kazuo KANNO ◽  
Yuki MOROKUMA ◽  
Toru TATENO ◽  
Yuki HIRONO ◽  
Kazuhiro TAKI ◽  
...  
Keyword(s):  
Olfactory Neuroblastoma ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth

scholarly journals Ectopic adrenocorticotropic hormone syndrome associated with olfactory neuroblastoma: acquirement of adrenocorticotropic hormone expression during disease course as shown by serial immunohistochemistry examinations

2018 ◽  
Vol 46 (11) ◽  
pp. 4760-4768 ◽  
Author(s):  
Manabu Kadoya ◽  
Masafumi Kurajoh ◽  
Akio Miyoshi ◽  
Takuhito Shoji ◽  
Tomonori Terada ◽  
...  
Keyword(s):  
Adrenocorticotropic Hormone ◽  
Clinical Course ◽  
Elevated Serum ◽  
Malignant Neoplasm ◽  
Olfactory Neuroblastoma ◽  
Serum Levels ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
First Case ◽  
Ectopic Adrenocorticotropic Hormone

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.

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

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.

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

2019 ◽  
Author(s):  
Hironori Abe ◽  
Hirotsugu Suwanai ◽  
Takuya Ishikawa ◽  
Fumiyoshi Yakou ◽  
Natsuko Hara ◽  
...  
Keyword(s):  
Rare Case ◽  
Olfactory Neuroblastoma ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth

Adrenocortical Ultrastructure in Ectopic Acth Syndrome

1976 ◽  
Vol 34 ◽  
pp. 238-239
Author(s):  
K. Kovacs ◽  
E. Horvath ◽  
W. Singer
Keyword(s):  
Adrenal Cortex ◽  
Zona Glomerulosa ◽  
Ectopic Acth Syndrome ◽  
Pituitary Neoplasms ◽  
Electron Microscopic ◽  
Adrenocortical Cells ◽  
Cell Hyperplasia ◽  
Ectopic Acth ◽  
Ectopic Acth Production ◽  
Microscopic Findings

Secretion of ACTH by non-pituitary neoplasms is recognized with increasing frequency. While the clinical and biochemical changes associated with ectopic ACTH production have been extensively studied recently, relatively little attention was focused on the morphology of the adrenal cortex and, to our knowledge, the fine structure of the adrenocortical cells in cases of ectopic ACTH syndrome has not been described so far. We report here the electron microscopic findings in the adrenal cortex of a 50-year-old man with a pancreatic apudoma. The patient showed the characteristic clinical and biochemical features of ectopic ACTH syndrome and because of extensive hypercorticism, underwent bilateral adrenalectomy.By light microscopy, the adrenal cortices showed extensive compact cell hyperplasia and lipid depletion. The zona glomerulosa was present in small foci and, except for a few places, fasciculata cells were noted under the fibrous capsule.

Electron Microscopic investigation of crooke’s change in the pituitaries of patients with hypercorticism

1989 ◽  
Vol 47 ◽  
pp. 848-849
Author(s):  
E. Horvath ◽  
K. Kovacs ◽  
L. Stefaneanu ◽  
N. Losinski
Keyword(s):  
Nucleolar Organizer ◽  
Microscopic Investigation ◽  
Electron Microscopic Investigation ◽  
Ectopic Acth Syndrome ◽  
Nucleolar Organizer Regions ◽  
Electron Microscopic ◽  
Human Pituitary ◽  
Ectopic Acth ◽  
Crooke’S Hyalinization

Human pituitary corticotropins have unique morphologic markers: bundles of type-1 filaments, measuring approximately 70 A in width and representing cytokeratin. The extreme ring-like accumulation of type-1 filaments, known as Crooke's hyalinization, signals functional suppression of the corticotropins and occurs in endogenous and exogenous glucocorticoid excess, caused by ACTH-secreting pituitary adenoma, glucocorticoid secreting adrenocortical tumor, ectopic ACTH-syndrome and administration of pharmacologic doses of glucocorticoids. Cells of autonomous corticotroph adenomas usually do not show Crooke's hyalin change. A minority of these tumors, however, retains sensitivity to the negative feed-back effect of elevated blood glucocorticoid levels and display typical Crooke’s change.In the present study pituitary corticotropins in various phases of Crooke's hyalinization were investigated in patients with glucocorticoid excess of various origin, applying histology, immunocytochemistry, count of argyrophilic nucleolar organizer regions (AgNOR), and transmission electron microscopy.

Abstract #21: Ectopic ACTH Syndrome from Cervical Cancer

2003 ◽  
Vol 9 ◽  
pp. 8
Author(s):  
Kashif M. Munir ◽  
Richard B. Horenstein
Keyword(s):  
Cervical Cancer ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth
Sign in / Sign up

Export Citation Format

Share Document