Ectopic ACTH Cushing’s Syndrome: V3 Vasopressin Receptor But Not CRH Receptor Gene Expression in a Pulmonary Carcinoid Tumor

1998 ◽  
Vol 50 (4) ◽  
pp. 226-231 ◽  
Author(s):  
Valdo Chabot ◽  
Yves de Keyzer ◽  
Sandra Gebhard ◽  
Antoine Uské ◽  
Angelika Bischof-Delaloye ◽  
...  
Keyword(s):  
Gene Expression ◽  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Receptor Gene ◽  
Cushing's Syndrome ◽  
Vasopressin Receptor ◽  
Ectopic Acth ◽  
Pulmonary Carcinoid ◽  
Receptor Gene Expression

Ectopic Cushing's Syndrome Secondary to Pulmonary Carcinoid Tumor

2013 ◽  
Vol 95 (5) ◽  
pp. 1797-1799 ◽  
Author(s):  
Shahriar Hashemzadeh ◽  
Atabak Asvadi Kermani ◽  
Akbar Ali-Asgharzadeh ◽  
Moneireh Halimi ◽  
Mina Soleimani ◽  
...  
Keyword(s):  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Pulmonary Carcinoid ◽  
Ectopic Cushing’S Syndrome

scholarly journals Occult Carcinoid Causes Cushing’s Syndrome

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A594-A595
Author(s):  
Sabrina Meftali ◽  
Rebecca Unterborn ◽  
Amanda Gifford ◽  
Bankim Bhatt
Keyword(s):  
Weight Gain ◽  
Carcinoid Tumor ◽  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Pulmonary Nodule ◽  
Cushing's Syndrome ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Ectopic Cushing’S Syndrome

Abstract Introduction: Lung neuroendocrine tumors (NETs) are a common cause of ectopic ACTH-secretion. Only 3% of NETs cause Cushing’s syndrome1; the majority are small and may be radiographically occult. Additionally, the responses to dexamethasone, metyrapone, and oCRF can be indistinguishable from that observed in pituitary Cushing’s disease2. These challenges lead to misdiagnosis and unnecessary procedures. Case Description: 45-year-old male presented for resection of right-sided pulmonary carcinoid tumor. He was diagnosed with Cushing’s syndrome in 2011 when he experienced uncontrolled hypertension and excessive weight gain with elevations in cortisol and ACTH levels. He underwent transsphenoidal pituitary resection in May 2011 for a possible pituitary microadenoma. Post-operative worsening hypertension, weight gain, and striae led to bilateral adrenalectomy in November 2011, complicated by retroperitoneal hemorrhage and resuscitation-induced pulmonary edema. On chest CT in early 2012, an 8 mm incidental right pulmonary nodule was documented. 8 years later, chest imaging for mild COVID-19 infection again revealed a right-sided pulmonary nodule. He underwent CT-guided biopsy of the nodule in July 2020 with pathology demonstrating carcinoid histology. Right lower lobectomy was performed. Pre-operative ACTH was 1673 pg/mL (normal: 10-60 pg/mL) and post-operative ACTH was 16 pg/mL. The resected tumor stained positive for ACTH, confirming that carcinoid tumor was the source of Cushing’s for the past decade. Conclusion: The diagnosis of ectopic Cushing’s syndrome can be elusive, leading to surgeries with significant morbidity, as seen in our patient. In one retrospective review, 14% of patients with Cushing’s had transsphenoidal resection before they were diagnosed with an ectopic source3. Ectopic Cushing’s syndrome can be a challenging diagnosis to make and a multidisciplinary approach with close collaboration between endocrinologist, radiologist, surgeon and pathologist may increase the diagnosis accuracy. Citations: 1Kamp, K, Alwani, R A, Korpershoek, E, Franssen, G., de Herder, W W, & Feelders, R A. (2016). Prevalence and clinical features of the ectopic ACTH syndrome in patients with gastroenteropancreatic and thoracic neuroendocrine tumors, European Journal of Endocrinology, 174(3), 271-280. Retrieved Jan 15, 2021, from https://eje.bioscientifica.com/view/journals/eje/174/3/271.xml 2Malchoff CD, Orth DN, Abboud C, Carney JA, Pairolero PC, Carey RM. Ectopic ACTH syndrome caused by a bronchial carcinoid tumor responsive to dexamethasone, metyrapone, and corticotropin-releasing factor. The American Journal of Medicine. 1988;84(4):760-764. doi:10.1016/0002-9343(88)90116-7 3Ioannis Ilias, David J. Torpy, Karel Pacak, Nancy Mullen, Robert A. Wesley, Lynnette K. Nieman,Cushing’s Syndrome Due to Ectopic Corticotropin Secretion: Twenty Years’ Experience at theNational Institutes of Health, The Journal of Clinical Endocrinology & Metabolism, Volume 90, Issue8, 1 August 2005, Pages 4955–4962, https://doi.org/10.1210/jc.2004-2527

scholarly journals Ectopic Acth-Producing Pulmonary Carcinoid: A Rare Case of Simulating the Cushing’s disease

2020 ◽  
Vol 3 (4) ◽  
pp. 01-04
Author(s):  
Manouchehr Nakhjavan
Keyword(s):  
Cushing’S Syndrome ◽  
Mediastinal Lymph Node ◽  
Left Lung ◽  
Cushing's Syndrome ◽  
Whole Body ◽  
High Dose ◽  
Ectopic Acth ◽  
Pulmonary Carcinoid ◽  
High Dose Dexamethasone ◽  
Free Cortisol

Excess amounts of blood glucocorticosteroids, either exogenous or endogenous, results in Cushing’s syndrome (CS). the current study presents a 45-year-old man, ACTH dependent Cushing’s syndrome. The basal cortisol and urine free cortisol level were significantly suppressed following high dose dexamethasone suppression test and pituitary MRI showed microadenoma with a size of less than 6 mm. After bilateral inferior petrosal sinus sampling, we investigated the ectopic source of ACTH production. The computed tomography revealed a mediastinal lymph node, and the whole body octreotide scan revealed octreotide avid lesion in the medial aspect of the left lung. The patient underwent left lung lobectomy and the pathological study indicated the carcinoid tumor of the left lung.

scholarly journals Association between tumoral GH-releasing peptide receptor type 1a mRNA expression and in vivo response to GH-releasing peptide-6 in ACTH-dependent Cushing's syndrome patients

2008 ◽  
Vol 158 (5) ◽  
pp. 605-613 ◽  
Author(s):  
Marcio Carlos Machado ◽  
Sandra Valeria de Sa ◽  
Maria Lucia Correa-Giannella ◽  
Ricardo Rodrigues Giorgi ◽  
Maria Adelaide Albergaria Pereira ◽  
...  
Keyword(s):  
Mrna Expression ◽  
Cushing’S Syndrome ◽  
Receptor Gene ◽  
Rna Extraction ◽  
Cushing's Syndrome ◽  
Receptor Type ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Peptide Receptor

ObjectiveGH secretagogues (GHS) produce exaggerated ACTH and cortisol responses in Cushing's disease (CD) patients, attributable to their direct action on GH-releasing peptide receptor type 1a (GHSR-1a). However, there are no studies correlating the in vivo response to GHS and GHSR-1a mRNA expression in ACTH-dependent Cushing's syndrome (CS) patients. The aim of this study is to correlate the patterns of ACTH and cortisol response to GH-releasing peptide-6 (GHRP-6) to GHSR-1a expression in ACTH-dependent CS patients.DesignProspective study in a tertiary referral hospital center. Fifteen CD patients and two ectopic ACTH syndrome (EAS) patients were studied.MethodsTumor fragments were submitted to RNA extraction, and GHSR-1a expression was studied through real-time qPCR and compared with normal tissue samples. The patients were also submitted to desmopressin test and vasopressin receptor type 1B (AVPR1B) mRNA analysis by qPCR.ResultsGHSR-1a expression was similar in normal pituitary samples and in corticotrophic tumor samples. GHSR-1a expression was higher in patients (CD and EAS) presenting in vivo response to GHRP-6. Higher expression of AVPR1B was observed in the EAS patients responsive to desmopressin, as well as in corticotrophic tumors, as compared with normal pituitary samples, but no correlation between AVPR1B expression and response to desmopressin was observed in the CD patients.ConclusionsOur results revealed a higher expression of GHSR-1a in the ACTH-dependent CS patients responsive to GHRP-6, suggesting an association between receptor gene expression and in vivo response to the secretagogue in both the CD and the EAS patients.

Cushing's syndrome and nocardiosis associated with a pulmonary carcinoid tumor: Report of a case and review of the literature

2010 ◽  
Vol 39 (5) ◽  
pp. 359-362 ◽  
Author(s):  
Brian J. Sutton ◽  
Graham E. Parks ◽  
Cyrus K. Manavi ◽  
Elizabeth L. Palavecino ◽  
Kim R. Geisinger
Keyword(s):  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Review Of The Literature ◽  
Pulmonary Carcinoid

scholarly journals Pulmonary embolism as the presenting symptom and a confounder in ACTH-secreting bronchial carcinoid

2019 ◽  
Vol 2019 ◽  
Author(s):  
Wei Yang ◽  
David Pham ◽  
Aren T Vierra ◽  
Sarah Azam ◽  
Dorina Gui ◽  
...  
Keyword(s):  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Lung Nodule ◽  
Cushing's Syndrome ◽  
Ectopic Acth Syndrome ◽  
List Type ◽  
Bronchial Carcinoid ◽  
Pulmonary Emboli ◽  
Ectopic Acth ◽  
Acth Secreting

Summary Ectopic ACTH-secreting pulmonary neuroendocrine tumors are rare and account for less than 5% of endogenous Cushing’s syndrome cases. We describe an unusual case of metastatic bronchial carcinoid tumor in a young woman presenting with unprovoked pulmonary emboli, which initially prevented the detection of the primary tumor on imaging. The source of ectopic ACTH was ultimately localized by a Gallium-DOTATATE scan, which demonstrated increased tracer uptake in a right middle lobe lung nodule and multiple liver nodules. The histological diagnosis was established based on a core biopsy of a hepatic lesion and the patient was started on a glucocorticoid receptor antagonist and a somatostatin analog. This case illustrates that hypercogulability can further aggravate the diagnostic challenges in ectopic ACTH syndrome. We discuss the literature on the current diagnosis and management strategies for ectopic ACTH syndrome. Learning points: In a young patient with concurrent hypokalemia and uncontrolled hypertension on multiple antihypertensive agents, secondary causes of hypertension should be evaluated. Patients with Cushing’s syndrome can develop an acquired hypercoagulable state leading to spontaneous and postoperative venous thromboembolism. Pulmonary emboli may complicate the imaging of the bronchial carcinoid tumor in ectopic ACTH syndrome. Imaging with Gallium-68 DOTATATE PET/CT scan has the highest sensitivity and specificity in detecting ectopic ACTH-secreting tumors. A combination of various noninvasive biochemical tests can enhance the diagnostic accuracy in differentiating Cushing’s disease from ectopic ACTH syndrome provided they have concordant results. Bilateral inferior petrosal sinus sampling remains the gold standard.

QUANTITATIVE ESTIMATION OF URINARY PREGNANETRIOL, PREGNANETRIOLONE, TETRAHYDRO S AND Δ5-PREGNENETRIOL IN THE INVESTIGATION OF ADRENOCORTICAL FUNCTION

1969 ◽  
Vol 60 (4) ◽  
pp. 657-668 ◽  
Author(s):  
Frances J. Thomas ◽  
A. W. Steinbeck
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Cushing’S Syndrome ◽  
Adrenocortical Carcinoma ◽  
Quantitative Estimation ◽  
Cushing's Syndrome ◽  
Adrenocortical Function ◽  
Adrenal Hyperplasia ◽  
Ectopic Acth ◽  
Modified Method ◽  
Idiopathic Hirsutism

ABSTRACT A modified method for the estimation of urinary pregnanetriol, pregnanetriol, pregnanetriolone, Δ5-pregnenetriol and tetrahydro S was investigated. The steroids, separated by chromatography, were measured quantitatively, tetrahydro S by reaction with blue tetrazolium and the other three as acetaldehydogenic substances. The excretion of these steroids was studied in suspected instances of adrenal and/or ovarian disease. Urinary pregnanetriol and tetrahydro S levels were normal in Cushing's syndrome, secondary adrenocortical carcinoma, the Stein-Leventhal syndrome, idiopathic hirsutism and hypertension. Tetrahydro S was doubtfully elevated in an instance of ectopic ACTH Cushing's syndrome. Pregnanetriol excretion was elevated in untreated cases of congenital adrenal hyperplasia and after treatment in some. Pregnanetriolone was found in all patients with congenital adrenal hyperplasia, including those receiving cortisone. Pregnanetriolone was also detected in Cushing's syndrome, secondary adrenocortical carcinoma, the Stein-Leventhal syndrome, ectopic ACTH Cushing's syndrome and suspected congenital adrenal hyperplasia. Δ5-pregnenetriol was present as a glucuronide in the Stein-Leventhal syndrome and idiopathic hirsutism. The diagnostic implications of these results are discussed.

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