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ectopic ACTH

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https://www.readbyqxmd.com/read/28816283/-surgery-treatment-of-ectopic-adrenocorticotrophic-hormone-syndrome
#1
H Fan, H Z Li, W F Xu, Z G Ji, Y S Zhang
OBJECTIVE: To investigation the diagnosis and treatment of ectopic adrenocorticotrophic hormone (ACTH) syndrome. METHODS: The clinical characters of 57 cases of ecotopic ACTH syndrome from Jan. 1996 to Dec. 2016 were collected and analyzed. The 57 cases included 32 males and 25 females. The age ranged from 11 to 68 years (average 32 years). ACTH levels significantly increased from 16.5 to 365.6 pmol/L, with average 77.6 pmol/L (normal range <10.1 pmol/L). The pituitary MRI did not found lesions...
August 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/28710116/expression-and-mutational-status-of-usp8-in-tumors-causing-ectopic-acth-secretion-syndrome
#2
Luis Gustavo Pérez-Rivas, Andrea Oßwald, Thomas Knösel, Kristin Lucia, Christian Schaaf, Michael Hristov, Julia Fazel, Thomas Kirchner, Felix Beuschlein, Martin Reincke, Marily Theodoropoulou
The ectopic ACTH secretion syndrome (EAS) is the clinical consequence of the paraneoplastic secretion of ACTH by non-pituitary tumors. The mechanisms responsible for the pathogenesis of these tumors are still unknown. Recently, we and others detected hotspot heterozygous driver mutations in the gene coding for the ubiquitin-specific protease 8 (USP8) in almost half of ACTH-secreting pituitary corticotroph tumors causing Cushing's disease. Mutant USP8 was also found to stimulate proopiomelanocortin (POMC) transcription and ACTH synthesis...
July 14, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28680643/a-rare-case-of-an-acth-crh-co-secreting-midgut-neuroendocrine-tumor-mimicking-cushing-s-disease
#3
Regina Streuli, Ina Krull, Michael Brändle, Walter Kolb, Günter Stalla, Marily Theodoropoulou, Annette Enzler-Tschudy, Stefan Bilz
Ectopic ACTH/CRH co-secreting tumors are a very rare cause of Cushing's syndrome and only a few cases have been reported in the literature. Differentiating between Cushing's disease and ectopic Cushing's syndrome may be particularly difficult if predominant ectopic CRH secretion leads to pituitary corticotroph hyperplasia that may mimic Cushing's disease during dynamic testing with both dexamethasone and CRH as well as bilateral inferior petrosal sinus sampling (BIPSS). We present the case of a 24-year-old man diagnosed with ACTH-dependent Cushing's syndrome caused by an ACTH/CRH co-secreting midgut NET...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28642733/a-case-of-pulmonary-carcinoid-tumor-with-a-superimposed-aspergilloma-presenting-as-a-covert-ectopic-adrenocorticotropic-hormone-syndrome
#4
Kyoung Jin Kim, Ji Hee Yu, Nan Hee Kim, Young Hye Kim, Young Sik Kim, Ji A Seo
Ectopic adrenocorticotropic hormone (ACTH) syndrome is a challenging diagnosis only responsible for approximately 10% of Cushing syndrome cases. It has been associated with a variety of benign and malignant tumors including a carcinoid tumor accompanied by aspergilloma in our case that was significantly difficult to be detected. We report a patient over 70 years old with uncontrolled hypertension and hypokalemia presenting with generalized edema. Laboratory results revealed ACTH-dependent Cushing syndrome, but imaging studies did not show any discrete lesions secreting ACTH...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28639924/cushing-syndrome-diagnostic-workup-and-imaging-features-with-clinical-and-pathologic-correlation
#5
REVIEW
Nicolaus A Wagner-Bartak, Ali Baiomy, Mouhammed Amir Habra, Shalini V Mukhi, Ajaykumar C Morani, Brinda R Korivi, Steven G Waguespack, Khaled M Elsayes
OBJECTIVE: Cushing syndrome (CS) is a constellation of clinical signs and symptoms resulting from chronic exposure to excess cortisol, either exogenous or endogenous. Exogenous CS is most commonly caused by administration of glucocorticoids. Endogenous CS is subdivided into two types: adrenocorticotropic hormone (ACTH) dependent and ACTH independent. CONCLUSION: Cushing disease, which is caused by a pituitary adenoma, is the most common cause of ACTH-dependent CS for which pituitary MRI can be diagnostic, with bilateral inferior petrosal sinus sampling useful in equivocal cases...
July 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28614036/an-individualized-approach-to-the-evaluation-of-cushing-syndrome
#6
Susmeeta T Sharma
Cushing syndrome (CS) is caused by chronic exposure to excess glucocorticoids. Early recognition and treatment of hypercortisolemia can lead to decreased morbidity and mortality. The diagnosis of CS and thereafter, establishing the cause can often be difficult, especially in patients with mild and cyclic hypercortisolism. Surgical excision of the cause of excess glucocorticoids is the optimal treatment for CS. Medical therapy (steroidogenesis inhibitors, medications that decrease adrenocorticotropic hormone [ACTH] levels or glucocorticoid antagonists) and pituitary radiotherapy may be needed as adjunctive treatment modalities in patients with residual, recurrent or metastatic disease, in preparation for surgery, or when surgery is contraindicated...
June 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28614007/ectopic-cushing-syndrome-a-10-year-experience-from-a-tertiary-care-center-in-southern-india
#7
Samantha Sathyakumar, Thomas Vizhalil Paul, Hesargatta Shyamsunder Asha, Birla Roy Gnanamuthu, M J Paul, Deepak Thomas Abraham, Simon Rajaratnam, Nihal Thomas
OBJECTIVE: Ectopic adrenocorticotropic hormone (ACTH) secretion is a less common cause of Cushing syndrome and is seen in 5 to 10% of cases with endogenous hypercortisolemia. We hereby describe our experience of patients with ectopic ACTH syndrome, who have been managed over the past 10 years at a tertiary care center in Southern India. METHODS: The inpatient and outpatient records of patients from 2006 to 2015 were retrospectively reviewed. The clinical features, clinical history, biochemical values, imaging features, including radiologic findings and positron emission tomography scans, management, details of follow-up, and outcomes, were documented...
August 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28590513/diagnostic-accuracy-of-increased-urinary-cortisol-cortisone-ratio-to-differentiate-acth-dependent-cushing-s-syndrome
#8
Filippo Ceccato, Laura Trementino, Mattia Barbot, Giorgia Antonelli, Mario Plebani, Luca Denaro, Daniela Regazzo, Federico Rea, Anna Chiara Frigo, Carolina Concettoni, Marco Boscaro, Giorgio Arnaldi, Carla Scaroni
BACKGROUND AND AIM: Differential diagnosis between Cushing's Disease (CD) and Ectopic ACTH Syndrome (EAS) may be a pitfall for endocrinologists. The increasing use in clinical practice of chromatography and mass spectrometry improves the measurement of urinary free cortisol (UFF) and cortisone (UFE). We have recently observed that cortisol to cortisone ratio (FEr) was higher in a small series of EAS; in this paper we collected a larger number of ACTH-dependent CS to study the role of FEr to characterize the source of corticotropin secretion...
June 7, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28584167/severe-cushing-s-syndrome-due-to-small-cell-prostate-carcinoma-a-case-and-review-of-literature
#9
REVIEW
M S Elston, V B Crawford, M Swarbrick, M S Dray, M Head, J V Conaglen
Cushing's syndrome (CS) due to ectopic adrenocorticotrophic hormone (ACTH) is associated with a variety of tumours most of which arise in the thorax or abdomen. Prostate carcinoma is a rare but important cause of rapidly progressive CS. To report a case of severe CS due to ACTH production from prostate neuroendocrine carcinoma and summarise previous published cases. A 71-year-old male presented with profound hypokalaemia, oedema and new onset hypertension. The patient reported two weeks of weight gain, muscle weakness, labile mood and insomnia...
July 2017: Endocrine Connections
https://www.readbyqxmd.com/read/28567290/acth-secreting-medullary-thyroid-cancer-a-case-series
#10
Ayanthi A Wijewardene, Sarah J Glastras, Diana L Learoyd, Bruce G Robinson, Venessa H M Tsang
Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour that originates from the parafollicular cells of the thyroid gland. The most common presentation of MTC is with a single nodule; however, by the time of diagnosis, most have spread to the surrounding cervical lymph nodes. Cushing's syndrome is a rare complication of MTC and is due to ectopic adrenocorticotrophic hormone (ACTH) secretion by tumour cells. Cushing's syndrome presents a challenging diagnostic and management issue in patients with MTC...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28532906/first-report-of-coexistence-of-two-ectopic-pituitary-tumors-rathke-cleft-cyst-and-silent-adrenocorticotropic-hormone-adenoma
#11
Mirza Pojskić, Blazej Zbytek, Neal S Beckford, Frederick A Boop, Kenan I Arnautović
BACKGROUND: Rathke cleft cysts (RCCs) and pituitary adenomas (PAs) are thought to have a common embryonic ancestry; however, PAs with a concomitant RCC inside the sella turcica are rarely observed. Ectopic pituitary tumors are also rare. CASE DESCRIPTION: We present the case of a 65-year-old woman with an ectopic RCC in the sphenoid sinus and outside the sella turcica concomitant with an adrenocorticotropic hormone (ACTH)-staining, clinically silent PA. The patient had headache but no endocrine or visual disturbances...
August 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28512418/cushing-s-syndrome-cortisol-and-cognitive-competency-a-case-report
#12
Neil Oronsky, Bennett Thilagar, Carolyn M Ray, Scott Caroen, Michelle M C Lybeck, Lindsey Ferry, Ronald A Voves, Bryan Oronsky
Glucocorticoids are associated with immunosuppression and neuropsychiatric complications. We describe the case of a carcinoid patient with Cushing's syndrome (CS) and neurocognitive impairment due to ectopic ACTH production who developed sepsis and died because of his family's decision to withdraw antibiotic treatment. This report is presented to illustrate the importance of advanced-care planning in patients with CS.
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28512417/a-case-of-paraneoplastic-cushing-syndrome-presenting-as-hyperglycemic-hyperosmolar-nonketotic-syndrome
#13
Christina E Brzezniak, Nicole Vietor, Patricia E Hogan, Bryan Oronsky, Bennett Thilagar, Carolyn M Ray, Scott Caroen, Michelle Lybeck, Neil Oronsky, Corey A Carter
Carcinoid tumors are neuroendocrine tumors that mainly arise in the gastrointestinal tract, lungs, and bronchi. Bronchopulmonary carcinoids have been associated with Cushing syndrome, which results from ectopic adrenocorticotrophic hormone (ACTH) secretion. We report the case of a 65-year-old man, a colonel in the US Air Force, with metastatic bronchopulmonary carcinoid tumors treated on a clinical trial who was hospitalized for complaints of increasing thirst, polydipsia, polyuria, weakness, and visual changes...
January 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28449502/bronchopulmonary-carcinoid-with-a-single-lymph-node-metastasis-causing-ectopic-cushing-s-syndrome
#14
Nilgün Büyükakyüz, Sven Hillinger, Walter Weder, Wolfgang Jungraithmayr
Bronchial carcinoid tumors (BCTs) represent a subgroup of bronchopulmonary neuroendocrine tumors which have the ability to produce neuropeptides. Merely 2% of BCTs secrete ACTH and less than 1% of patients with Cushing's syndrome (CS) have a BCT. Typical BCTs rarely metastasize. We here present a patient with ectopic CS caused by a typical BCT, which metastasized into a lymph node. This case suggests that the diagnostic work up of CS should be wide and include ectopic ACTH-secreting BCT, and that ACTH secretion can be correlated to a more aggressive type of typical BCTs...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28433999/circadian-plasma-cortisol-measurements-reflect-severity-of-hypercortisolemia-in-children-with-different-etiologies-of-endogenous-cushing-syndrome
#15
Amit Tirosh, Maya B Lodish, Charalampos Lyssikatos, Elena Belyavskaya, Georgios Z Papadakis, Constantine A Stratakis
BACKGROUND: The utility of circadian cortisol variation in estimating the degree of hypercortisolemia in different forms of endogenous Cushing syndrome (CS) has not been evaluated in children yet. METHODS: A retrospective cohort study, including children who underwent surgery due to CS (n = 115), was divided into children with a pituitary adenoma (Cushing disease) (n = 88), primary adrenal CS (n = 21), or ectopic adrenocorticotropin- or corticotropin-releasing hormone (ACTH-/CRH)-secreting tumors (n = 6)...
2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28377460/diagnostic-tests-for-cushing-s-syndrome-differ-from-published-guidelines-data-from-ercusyn
#16
MULTICENTER STUDY
Elena Valassi, Holger Franz, Thierry Brue, Richard A Feelders, Romana Netea-Maier, Stylianos Tsagarakis, Susan M Webb, Maria Yaneva, Martin Reincke, Michael Droste, Irina Komerdus, Dominique Maiter, Darko Kastelan, Philippe Chanson, Marija Pfeifer, Christian J Strasburger, Miklós Tóth, Olivier Chabre, Antoine Tabarin, Michal Krsek, Carmen Fajardo, Marek Bolanowski, Alicia Santos, John A H Wass, Peter J Trainer
OBJECTIVE: To evaluate which tests are performed to diagnose hypercortisolism in patients included in the European Registry on Cushing's syndrome (ERCUSYN), and to examine if their use differs from the current guidelines. PATIENTS AND METHODS: We analyzed data on the diagnostic tests performed in 1341 patients with Cushing's syndrome (CS) who have been entered into the ERCUSYN database between January 1, 2000 and January 31, 2016 from 57 centers in 26 European countries...
May 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28343156/ectopic-acth-producing-large-cell-neuroendocrine-pancoast-tumour-presenting-as-horner-syndrome
#17
Rajanshu Verma, Alexandra Lambert, Harold H Katz, Scott J Benson
We present an interesting case where a patient is presented with a droopy left eyelid (as part of Horner syndrome) and Cushingoid features which were a result of a Pancoast tumour (apical lung tumour in superior pulmonary sulcus) involving the left lung. This tumour was secreting ectopic adrenocorticotropic hormone (ACTH), a paraneoplastic endocrine phenomenon, which resulted in Cushing syndrome symptomatology. Though most ectopic ACTH-producing lung cancers are either small cell or carcinoid tumours, this was in fact a large cell neuroendocrine cancer (LCNEC)...
March 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28328532/cushing-s-syndrome-in-infancy-due-to-ectopic-acth-secretion-by-a-sacro-coccygeal-teratoma
#18
Marta Rydzewska, Maryna Krawczuk-Rybak, Adrianna Zajkowska, Natalia Jurczuk, Dariusz Polnik, Mieczysław Szalecki, Elżbieta Moszczyńska, Martin O Savage, Artur Bossowski
BACKGROUND: Adenocorticotropic hormone (ACTH)-dependent Cushing's syndrome in infancy is extremely rare. We describe the case of a sacro-coccygeal ectopic ACTH-secreting immature teratoma in an infant who also presented the triad of defects characteristic of Currarino syndrome. CASE PRESENTATION: A girl was born with a large immature teratoma in the sacro-coccygeal region associated with anal atresia. At the age of 7 days, the concentration of α-fetoprotein (AFP) was above the age-specific normal range...
April 1, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28317048/acth-producing-neuroendocrine-tumor-of-the-pancreas-a-case-report-and-literature-review
#19
Justin Byun, Sung Hyun Kim, Hyang Sook Jeong, Yumie Rhee, Woo Jung Lee, Chang Moo Kang
Tumors that arise from the endocrine pancreas, or the islets of pancreas, are called pancreatic neuroendocrine tumors (NETs). Pancreatic NET have an incidence of <0.1 per one million persons, and can lead to secretion of ectopic adrenocorticotropic hormone (ACTH). Herein, we presented a case of patient with Cushing's syndrome as a result of ACTH-producing pancreatic NET, who underwent successful laparoscopic distal pancreatosplenectomy. A 40-year-old Korean female patient with ophthalmologic discomfort, osteoporosis, and unexplained hypokalemia was admitted to our hospital...
February 2017: Annals of Hepato-Biliary-Pancreatic Surgery
https://www.readbyqxmd.com/read/28250304/pneumocystis-pneumonia-concomitant-with-ectopic-acth-syndrome-caused-by-a-large-cell-neuroendocrine-carcinoma-of-the-thymus
#20
Naohiro Oda, Nobuaki Miyahara, Masahiro Tabata, Daisuke Minami, Kiichiro Ninomiya, Arihiko Kanehiro, Motoshi Komatsubara, Kenichi Inagaki, Mitsune Tanimoto, Katsuyuki Kiura
We herein report the case of a 44-year-old man who was diagnosed with pneumocystis pneumonia (PCP) concomitant with ectopic adrenocorticotropic hormone (ACTH) syndrome, which had been caused by a large cell neuroendocrine carcinoma of the thymus. Chest computed tomography revealed ground-glass opacities in the lungs. PCP was diagnosed by a polymerase chain reaction with bronchoalveolar lavage. The levels of cortisol were slowly corrected with an adrenal enzyme inhibitor, and the exacerbation of PCP was successfully avoided...
2017: Internal Medicine
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