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 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.

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

scholarly journals An Ectopic ACTH Secreting Metastatic Parotid Tumour

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Thomas Dacruz ◽  
Atul Kalhan ◽  
Majid Rashid ◽  
Kofi Obuobie
Keyword(s):  
Neuroendocrine Tumours ◽  
Case Reports ◽  
Surgical Excision ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Delay In Diagnosis ◽  
Parotid Tumour ◽  
Original Tumour ◽  
Ectopic Adrenocorticotropic Hormone ◽  
Acth Secreting

A 60-year old woman presented with features of Cushing’s syndrome (CS) secondary to an ectopic adrenocorticotropic hormone (ACTH) secreting metastatic parotid tumour 3 years after excision of the original tumour. She subsequently developed fatal intestinal perforation and unfortunately died despite best possible medical measures. Ectopic ACTH secretion accounts for 5–10% of all patients presenting with ACTH dependent hypercortisolism; small cell carcinoma of lung (SCLC) and neuroendocrine tumours (NET) account for the majority of such cases. Although there are 4 previous case reports of ectopic ACTH secreting salivary tumours in literature, to our knowledge this is the first published case report in which the CS developed after 3 years of what was deemed as a successful surgical excision of primary salivary tumour. Our patient initially had nonspecific symptoms which may have contributed to a delay in diagnosis. Perforation of sigmoid colon is a recognised though underdiagnosed complication associated with steroid therapy and hypercortisolism. This case demonstrates the challenges faced in diagnosis as well as management of patients with CS apart from the practical difficulties faced while trying to identify source of ectopic ACTH.

scholarly journals Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization

2021 ◽  
Vol 93 (10) ◽  
pp. 1171-1178
Author(s):  
Olga O. Golounina ◽  
Zhanna E. Belaya ◽  
Liudmila Ya. Rozhinskaya ◽  
Evgeniya I. Marova ◽  
Michail Yu. Pikunov ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Fatal Outcome ◽  
Distant Metastases ◽  
Multiple Organ ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Results Of Treatment ◽  
Single Center Study ◽  
Pancreatic Net

Aim. To study the clinical, biochemical characteristics, treatment results and follow-up of patients with ectopic ACTH syndrome EAS (ACTH adrenocorticotropic hormone ). Materials and methods. A retrospective, observational, single-center study of 130 patients with EAS. Demographic information of patients, medical history, results of laboratory and instrumental investigations at the pre- and postoperative stages and follow-up of EAS were analyzed. Results. The mean age at the diagnosis ranged from 12 to 74 years (Me 40 years [28; 54]). The duration of the disease from the onset of symptoms to the verification of the diagnosis varied from 2 to 168 months (Me 17.5 months [7; 46]). Eighty-one (62,3%) patients had bronchopulmonary NET, 9 thymic carcinoid, 7 pancreatic NET, 5 pheochromocytoma, 1 cecum NET, 1 appendix carcinoid tumor, 1 medullary thyroid cancer and 25 (19.2%) had an occult source of ACTH. The median follow-up period of patients was 27 months [9.75; 61.0] with a maximum follow-up of 372 months. Currently, primary tumor was removed in 82 (63.1%) patients, bilateral adrenalectomy was performed in 23 (18%) patients, in 16 of them there was an occult source of ACTH-producing NET and in 7 patients in order to control hypercortisolism after non-successful surgical treatment. Regional and distant metastases were revealed in 25 (19.2%) patients. At the time of the last observation 59 (72%) patients were exhibited a full recovery, 12 (14.6%) had relapse of the disease and 26 (20%) died from multiple organ failure (n=18), pulmonary embolism (n=4), surgical complications (n=2), disseminated intravascular coagulation syndrome (n=1) or COVID-19 (n=1). Conclusion. In our cohort of patients bronchopulmonary NET are the most frequent cause of EAS (62.3%). Surgical treatment leads to remission of hypercortisolism in 72% cases; the proportion of relapse (14.6%) and fatal outcome (20%) remains frequent in EAS.

scholarly journals Possible pitfalls in the workup of ectopic ACTH secretion illustrated by four rare cases

2019 ◽  
Vol 12 (11) ◽  
pp. e231067
Author(s):  
Jorianne Boers ◽  
P Christine Oldenburg-Ligtenberg ◽  
Aline ME Stades ◽  
Mark JC van Treijen
Keyword(s):  
Case Report ◽  
Adrenocorticotropic Hormone ◽  
Progressive Disease ◽  
Diagnostic Workup ◽  
Morbidity And Mortality ◽  
Significant Morbidity ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Ectopic Adrenocorticotropic Hormone

In this case report, we highlight four different cases of ectopic adrenocorticotropic hormone (ACTH) secretion with different pitfalls in the diagnostic workup. Ectopic ACTH secretion (EAS) is an uncommon cause of Cushing’s syndrome that accompanies a variety of tumours. It is associated with significant morbidity and mortality. This underlines the importance of early and adequate diagnosis. We will review the causes of EAS and their presentation to increase awareness of this rare and progressive disease.

scholarly journals The role of 68Ga-DOTA derivatives PET-CT in patients with ectopic ACTH syndrome

2020 ◽  
Vol 9 (4) ◽  
pp. 337-345
Author(s):  
Filippo Ceccato ◽  
Diego Cecchin ◽  
Michele Gregianin ◽  
Giacomo Ricci ◽  
Cristina Campi ◽  
...  
Keyword(s):  
Small Cell Lung Carcinoma ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Ectopic Acth Secretion ◽  
Bronchial Carcinoids ◽  
Pet Ct ◽  
Pancreatic Net ◽  
Acth Secreting

Introduction and aim Ectopic ACTH secretion (EAS) is mostly secondary to thoracic/abdominal neuroendocrine tumours (NETs) or small cell-lung carcinoma (SCLC). We studied the diagnostic accuracy of CT with 68Ga-Dota derivatives (68Ga-SSTR) PET in localizing ACTH-secreting tumor in patients with EAS. Materials and methods 68Ga-SSTR-PET/CT was performed and compared with the nearest enhanced CT in 18 cases (16 primary and 2 recurrent neoplasms). Unspecific, indeterminate and false-positive uptakes were assessed using conventional imaging, follow-up or histology. Results We diagnosed 13 thoracic (9 primary and 2 recurrent bronchial carcinoids, 2 SCLCs) and 1 abdominal (pancreatic NET) tumors. Eight ACTH-secreting tumors were promptly identified at EAS diagnosis (’overt’, four pulmonary carcinoids with two recurrences and two SCLC); six EAS have been discovered during the subsequent follow-up (’covert’, five bronchial carcinoids and one pancreatic NET). At the time of EAS diagnosis, imaging was able to correctly detect the ACTH-secreting tumour in 8/18 cases (6 new diagnosis and 2 recurrences). During the follow-up, six out of initially ten ‘occult’ cases became ‘covert’. At last available follow-up, CT and 68Ga-SSTR-PET/CT were able to diagnose 11/18 and 12/18 ACTH-secreting tumours, respectively (11/14 and 12/14 considering only overt and covert cases, respectively). Four cases have never been localized by conventional or nuclear imaging (’occult EAS’), despite an average follow-up of 5 years. Conclusion The 68Ga-SSTR-PET/CT is useful in localizing EAS, especially to enhance positive prediction of the suggestive CT lesions and to detect occult neoplasms.

scholarly journals Adrenocorticotropic hormone-producing pheochromocytoma: analysis of clinical cases

2015 ◽  
Vol 12 (3) ◽  
pp. 46-52
Author(s):  
Evgeniya Ivanovna Marova ◽  
Iya Aleksandrovna Voronkova ◽  
Anastasiya Mikhaylovna Lapshina ◽  
Svetlana Dmitrievna Arapova ◽  
Nikolay Sergeevich Kuznetsov ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Cushing’S Syndrome ◽  
Adrenocorticotropic Hormone ◽  
Somatostatin Receptors ◽  
Pituitary Tumors ◽  
Cushing's Syndrome ◽  
Ectopic Acth Syndrome ◽  
Ki 67 ◽  
Ectopic Acth ◽  
Free Cortisol

Ectopic secretion of ACTH from non-pituitary tumors, referred to as ectopic ACTH syndrome (EAS), accounts for about 10–20% of Cushing’s syndrome (CS). Ectopic hormone-secreting pheochromocytomas (Pheo) are rare. The first publication of association between pheochromocytoma and Cushing’s syndrome by Roux is dated 1955. Pheochromocytoma represents a rare cause of hypercortisolism, accounting for less than 5 % of ectopic Cushing’s syndrome while less than 1 % of pheochromocytomas is accompanied by Cushing’s syndrome.We are reporting 4 cases of ACTH-secreting pheochromocytoma presenting as Cushing’s syndrome. Data from 4 patients were analysed. There were 4 women from 50 to 63 years old. All patients had a clinical presentation of hypercorticoidism. Their levels of adrenocorticotropic hormone in plasma, 24-hour urinary free cortisol and urinary catecholamine were high. Computed tomography scan of the abdomen in all cases revealed a mass in the left adrenal gland. Left sided adrenalectomy was performed under treatment with a-blocker doxazosin and b-blocker atenolol. Histological examination revealed in 3 cases – pheocromocytoma and in 1 case corticomedullary mixed tumor of the adrenal gland. Additional immunostaining (IHC)of these tumors showed positive immunostaining for chromogranin and ACTH. The IHC search for somatostatin receptors of subtype 2 and 5 (SSTR2, SSTR5) was performed in 3 cases and showed predominately expression SSTR2. The case index of Ki-67 ranged, from 0,5 to 4%. Biochemical signs of hypercortisolism rapidly began to disappear after surgery. Follow up of the patients during the next 2 years on average was with disease remission.

Adrenocorticotropin Production by a Mammary Carcinoma

1977 ◽  
Vol 35 ◽  
pp. 462-463
Author(s):  
K.S. McCarty ◽  
N.R. Wallace ◽  
W. Litaker ◽  
S. Wells ◽  
G. Eisenbarth
Keyword(s):  
Adrenocorticotropic Hormone ◽  
Small Cell ◽  
Hormone Production ◽  
Carcinoma Of The Breast ◽  
Ectopic Acth ◽  
Ultrastructural Studies ◽  
Subcutaneous Metastases ◽  
Ectopic Acth Production ◽  
The Right ◽  
Pituitary Carcinomas

The production of adrenocorticotropic hormone by non-pituitary carcinomas has been documented in several tumors, most frequently small cell carcinoma of the lung, islet cell carcinomas of the pancreas, thymomas and carcinoids. Electron microscopy of these tumors reveals typical membrane-limited "neurosecretory" granules. Confirmation of the granules as adrenocorticotropin (ACTH) requires the use of OsO4 as a primary fixative to give the characteristic cored granule appearance in conjunction with immunohistochemical demonstration of the hormone peptide. Because of the rarity of ectopic ACTH production by mammary carcinomas and the absence of appropriate ultrastructural studies in the two examples of such ectopic hormone production in the literature of which we are aware (1,2), we present biochemical and ultrastructural data from a carcinoma of the breast with apparent ACTH production.The patient had her primary tumor in the right breast in 1969. The tumor recurred as visceral and subcutaneous metastases in 1976 and again in 1977.

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.

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