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 An atypical case of ectopic ACTH syndrome in an adolescent boy

2020 ◽  
Vol 2020 (3) ◽  
Author(s):  
Shreya Sharma ◽  
Rajesh Joshi
Keyword(s):  
Abdominal Pain ◽  
Adrenocorticotropic Hormone ◽  
Neuroendocrine Tumours ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Atypical Case ◽  
Intermittent Abdominal Pain ◽  
Pancreatic Net ◽  
Pancreatic Neuroendocrine Tumours ◽  
Ectopic Adrenocorticotropic Hormone

Abstract Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is exceedingly rare in children and scarcely reported. Pancreatic neuroendocrine tumours (NETs) can rarely lead to secretion of ectopic ACTH. A 14-year-old boy presented with hyperpigmentation, hypertension and intermittent abdominal pain, and was diagnosed with endogenous hypercortisolism. An incidental pancreatic mass discovered on routine ultrasonogram (USG) revealed the source of ACTH. He underwent successful excision of the mass with resolution of hypercortisolism. The histopathology revealed a Pancreatic NET and immunohistochemistry was positive for ACTH stain.

scholarly journals Adrenocorticotropic Hormone-Dependent Cushing Syndrome Caused by an Olfactory Neuroblastoma

2019 ◽  
Vol 12 ◽  
pp. 117955141982583 ◽  
Author(s):  
Cristina Familiar ◽  
Ane Azcutia
Keyword(s):  
Adrenocorticotropic Hormone ◽  
Cushing Syndrome ◽  
Combined Therapy ◽  
Malignant Neoplasm ◽  
Olfactory Neuroblastoma ◽  
Large Mass ◽  
High Dose ◽  
Cushing Disease ◽  
Fractionated Radiotherapy ◽  
High Dose Dexamethasone

Olfactory neuroblastoma (ONB) is an unusual malignant neoplasm originating from the olfactory neuroepithelium. Secretion of adrenocorticotropic hormone (ACTH) from this tumor has been exceptionally reported. We describe a young man with resistant hypertension and a cushingoid phenotype. After hormonal confirmation of an ACTH-dependent Cushing syndrome, non-invasive dynamic tests were carried out to evaluate the cause of the ACTH source. Plasma cortisol decrease after a high-dose dexamethasone suppression test and cortisol increase after a desmopressin (DDAVP) stimulation test suggested a Cushing disease. A magnetic resonance image (MRI) of the brain and an Indium-111 octreotide scan revealed a large mass centered in the sphenoid sinus with lateral and posterior extension. An ACTH secreting ONB was confirmed with a trasnasal biopsy. Patient was offered a combined therapy with surgical resection and radiotherapy but refused surgery. The neoplasm was treated with neoadjuvant cisplatin-based chemotherapy followed by fractionated radiotherapy. Hypercortisolism initially improved with metyrapone but normocortisolism was only achieved after local control of the tumor with radiotherapy. Clinical presentation of ONB is usually related to local symptoms (as nasal obstruction and epistaxis) dependent on its ubication and extension. Cushing syndrome from ACTH production is a rare manifestation of ONB. This case also underlies the difficulties related to the interpretation of dynamic endocrine tests in Cushing syndrome.

Gastro-intestinal polypeptides in patients treated for medullary carcinoma of the thyroid

1984 ◽  
Vol 106 (1) ◽  
pp. 112-115 ◽  
Author(s):  
Bente Rasmusson
Keyword(s):  
Serum Gastrin ◽  
Clinical Course ◽  
Elevated Serum ◽  
Medullary Carcinoma ◽  
Serum Levels ◽  
High Serum ◽  
Serum Concentrations ◽  
Serum Calcitonin ◽  
Basal Serum ◽  
Previously Treated

Abstract. In 12 patients treated 2 to 58 months previously for medullary carcinoma of the thyroid, basal serum concentrations of calcitonin, gastrin, vasoactive intestinal polypeptide, glucagon, insulin, and pancreatic polypeptide were measured in search of any correlation between these and the clinical course of the disease. All patients had elevated serum calcitonin levels indicating present disease. One patient had increased serum concentrations of several hormones. Another had achlorhydria and high serum gastrin levels. No relationship between calcitonin and gastro-intestinal polypeptides was found in 11 patients. No correlations were found between serum levels of polypeptides and the occurrence of diarrhoea in 5 patients. It is concluded that gastro-intestinal polypeptides, which are produced by other apudomas, are not secreted in more than normal concentrations under basal conditions, by the majority of patients previously treated for medullary carcinoma of the thyroid.

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

CLINICAL HETEROGENEITY OF ECTOPIC ACTH SYNDROME: A LONG-TERM FOLLOW-UP STUDY

2020 ◽  
Vol 26 (12) ◽  
pp. 1435-1441
Author(s):  
Ana Laura Espinosa-de-los-Monteros ◽  
Claudia Ramírez-Rentería ◽  
Moisés Mercado
Keyword(s):  
Adrenocorticotropic Hormone ◽  
Cushing Syndrome ◽  
Neuroendocrine Neoplasm ◽  
Ectopic Acth Syndrome ◽  
Tumor Type ◽  
Ectopic Acth ◽  
Long Term Outcome ◽  
Free Cortisol

Objective: Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is a heterogeneous condition caused by neuroendocrine neoplasms (NENs) located in the lungs, thymus, or pancreas. Our purpose was to evaluate the long-term outcome of these patients. Methods: Retrospective study at a referral center. The charts of 164 patients with Cushing syndrome, followed at our center from 1993 to 2019, were analyzed. Results: EAS was found in 16 patients (9.75%, 9 women, mean age 36.01 years) who had been followed for a median of 72 months. The source of EAS was a NEN in 10 patients (8 bronchial and 2 thymic carcinoid tumors) and a mixed corticomedullary tumor, consisting of a pheochromocytoma and an adrenocortical carcinoma in 1 patient. In 2 of the 6 patients initially considered to have occult EAS, the source of the ACTH excess became apparent after adrenalectomy, whereas in the remaining 4 (25%) patients, it has remained occult. Of the 11 patients in whom resection of the NEN was attempted, 10 patients achieved an early remission (91%), but 4 (25%) of these patients had a recurrence during follow-up (biochemically and clinically silent in 2 patients). Three patients died (18.75%): the young woman with the mixed corticomedullary tumor, a man with a thymic NEN that evolved into a neuroendocrine (NE) carcinoma after 11 years of follow-up, and a woman with a bronchial NEN. Conclusion: The course of EAS varies according to tumor type and grade. Some patients have a protracted course, whereas others may evolve into neuroendocrine carcinomas. Abbreviations: ACTH = adrenocorticotropic hormone; CS = Cushing syndrome; CT = computed tomography; CV = coefficient of variation; EAS = ectopic ACTH syndrome; IQR = interquartile range; NEN = neuroendocrine neoplasm; SCCL = small cell carcinoma of the lung; TSS = transsphenoidal surgery; UFC = urinary free cortisol

Serum chromogranin A in the differential diagnosis of Cushing's syndrome

1994 ◽  
Vol 131 (6) ◽  
pp. 589-593 ◽  
Author(s):  
Frank RE Nobels ◽  
Wouter W de Herder ◽  
Dik J Kwekkeboom ◽  
Willy Coopmans ◽  
Andries Mulder ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Cushing’S Syndrome ◽  
Immunohistochemical Staining ◽  
Chromogranin A ◽  
Neuroendocrine Tumours ◽  
Elevated Serum ◽  
Serum Levels ◽  
Cushing's Syndrome ◽  
Ectopic Acth ◽  
Adrenocortical Tumour

Nobels FRE, de Herder WW, Kwekkeboom DJ, Coopmans W, Mulder A, Bouillon R, Lamberts SWJ. Serum chromogranin A in the differential diagnosis of Cushing's syndrome. Eur J Endocrinol 1994:131:589–93. ISSN 0804–4643 We evaluated whether measuring serum levels of chromogranin A, a marker of neuroendocrine tumours, could be useful in the differential diagnosis between pituitary, adrenal and ectopic causes of Cushing's syndrome. Thirty patients with Cushing's syndrome were studied. The localization of the tumours responsible was pituitary in 15, adrenal in 5 and ectopic in 10 patients. Serum concentrations of chromogranin A were measured in all patients. Petrosal sinus sampling for chromogranin A was performed in the cases with pituitary-dependent Cushing's syndrome. Immunohistochemical staining for chromogranin A was carried out on part of the tumour specimens. Slightly elevated serum levels of chromogranin A (range 223–262 μg/1) were detected in inferior petrosal sinus and peripheral venous samples from three patients with pituitary-dependent Cushing's syndrome. Serum chromogranin A showed no significant pituitary to peripheral gradient in these patients. Chromogranin A levels were not elevated in cases of adrenal Cushing's syndrome. Markedly elevated concentrations (range 270–13900 μg/1) were shown in seven of 10 patients with neuroendocrine tumours with ectopic adrenocorticotrophin (ACTH) and/or corticotrophin-releasing hormone (CRH) production. Widespread metastasis was present in all these cases. Subjects with "occult" carcinoid tumours, with limited spread, had normal chromogranin A levels Immunohistochemical staining for chromogranin A was positive in three out of five pituitary adenomas and in all neuroendocrine tumours with ectopic ACTH and/or CRH production, while it was negative in all adrenocortical tumour specimens. It is concluded that elevated serum levels of chromogranin A can serve as markers of neuroendocrine tumours with ectopic ACTH and/or CRH production. The circulating levels are dependent mainly on the size of the tumours. Serum chromogranin A is not useful in the diagnosis of so-called occult Cushing's syndrome, caused by ectopic ACTH and/or CRH secretion by small neuroendocrine tumours. F Nobels, Department of Endocrinology, Onze Lieve Vrouw Hospital, 164 Moorselbaan, 9300 Aalst, Belgium

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