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ectopic ACTH

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https://www.readbyqxmd.com/read/28098665/source-of-ectopic-acth-secretion-easily-identified-by-68ga-dotanoc-pet-ct
#1
Sofiullah Abubakar, Yaw Ampem Amoako, Tessa Kotze, Naima Tag
Malignant tumors account for most sources of ectopic ACTH Cushing syndrome (EA-CS). Early localization of the source and complete removal can be curative and also prevent metastasis. Diagnostic CT is known to perform better than PET/CT (low dose) in characterizing lung pathologies. However, bronchial carcinoids, a common source of EA-CS, may be difficult to detect on chest CT scan especially when it is small and located close to the hilar region. We present a case of EA-CS due to bronchial carcinoid, which was easily seen on Ga DOTANOC PET/CT after a diagnostic chest CT was reported as normal...
January 16, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28068878/long-term-control-of-hypercortisolism-by-vandetanib-in-a-medullary-thyroid-carcinoma-with-a-somatic-ret-mutation
#2
Anne-Cécile Paepegaey, Beatrix Cochand-Priollet, Estelle Louiset, Pierre Olivier Sarfati, Marco Alifano, Nelly Burnichon, Marie Bienvenu-Perrard, Najiba Lahlou, Léopoldine Bricaire, Lionel Groussin
CONTEXT: Medullary thyroid carcinomas (MTCs) complicated by ectopic Cushing's syndrome have a poor prognosis, partially due to the difficulties to control hypercortisolism by adrenal blocking drugs. Recent reports (including the initial follow-up of this patient) have suggested that tyrosine kinase inhibitors (TKIs) may be a therapeutic option due to an antisecretory action on ACTH. However, there is a lack of long-term follow-up studies. CASE DESCRIPTION: We report the case of a 58 year-old man with a MTC-related Cushing's syndrome resistant to a combination of several anticortisolic drugs...
January 9, 2017: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/28003168/endoscopic-endonasal-surgery-for-remission-of-cushing-disease-caused-by-ectopic-intracavernous-macroadenoma-case-report-and-literature-review
#3
Maria Koutourousiou, Welby I Winstead
BACKGROUND: Complete surgical resection of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the gold standard of treatment of Cushing disease. Ectopic location of these adenomas is an extremely rare condition that may compromise the diagnosis and surgical success. We present the first case of an ectopic intracavernous ACTH-secreting macroadenoma totally resected with endoscopic endonasal surgery (EES). CASE DESCRIPTION: A 36-year-old woman presented with Cushing syndrome...
December 18, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27977570/hypokalemia-associated-with-a-solitary-pulmonary-nodule-a-case-report
#4
Samira Saeian, Seiyed Mohammad Ali Ghayumi, Mesbah Shams
BACKGROUND: Differential diagnosis of hypokalemia and adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome often presents challenging in endocrinology and requires careful clinical, biochemical, radiological, and pathological investigations. Hypokalemia is a common abnormality and systematic approach is required to avoid delays in diagnosis of important underlying causes. CASE SUMMARY & CONCLUSION: A 49-year-old woman presented with moderate hypokalemia...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27935805/e2f1-mediated-human-pomc-expression-in-ectopic-cushing-s-syndrome
#5
Takako Araki, Ning-Ai Liu, Yukiko Tone, Daniel Cuevas-Ramos, Roy Heltsley, Masahide Tone, Shlomo Melmed
Cushing's syndrome is caused by excessive adrenocorticotropic hormone (ACTH) secretion derived from pituitary corticotroph tumors (Cushing disease) or from non-pituitary tumors (ectopic Cushing's syndrome). Hypercortisolemic features of ectopic Cushing's syndrome are severe, and no definitive treatment for paraneoplastic ACTH excess is available. We aimed to identify subcellular therapeutic targets by elucidating transcriptional regulation of the human ACTH precursor POMC (proopiomelanocortin) and ACTH production in non-pituitary tumor cells and in cell lines derived from patients with ectopic Cushing's syndrome...
November 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27928434/adrenalectomy-for-cushing-s-syndrome-do-s-and-don-ts
#6
REVIEW
Paduraru Dn, Nica A, Carsote M, Valea A
Aim. To present specific aspects of adrenalectomy for Cushing's syndrome (CS) by introducing well established aspects ("do's") and less known aspects ("don'ts"). Material and Method. This is a narrative review. Results. The "do's" for laparoscopic adrenalectomy (LA) are the following: it represents the "gold standard" for secretor and non-secretor adrenal tumors and the first line therapy for CS with an improvement of cardio-metabolic co-morbidities; the success rate depending on the adequate patients' selection and the surgeon's skills...
October 2016: Journal of Medicine and Life
https://www.readbyqxmd.com/read/27913610/whole-exome-sequencing-of-thymic-neuroendocrine-tumor-with-ectopic-acth-syndrome
#7
Yanli Li, Ying Peng, Xiuli Jiang, Yulong Cheng, Weiwei Zhou, Tingwei Su, Jing Xie, Xu Zhong, Dalong Song, Luming Wu, Liwen Fan, Min Li, Jie Hong, Weiqing Wang, Guang Ning, Yanan Cao
OBJECTIVE: Thymic neuroendocrine tumor is the second-most prevalent cause of ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS), which is a rare disease characterized by ectopic ACTH oversecretion from nonpituitary tumors. However, the genetic abnormalities of thymic neuroendocrine tumors with EAS remain largely unknown. We aim to elucidate the genetic abnormalities and identify the somatic mutations of potential tumor-related genes of thymic neuroendocrine tumors with EAS by whole exome sequencing...
February 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27903577/adrenal-cushing-syndrome-with-detectable-acth-from-an-unexpected-source
#8
Thinzar M Lwin, Nehal Galal, Shweta Gera, Jennifer L Marti
Mixed corticomedullary adrenal tumours (MCMT) are rare. We describe the second reported case of a male patient presenting with hypertension and Cushing syndrome with MCMT. A man aged 48 years presented with hypertension and signs of Cushing syndrome. 24-hour urine cortisol was elevated, with detectable adrenocorticotropic hormone (ACTH). A high-dose dexamethasone suppression test indicated an adrenal or ectopic Cushing syndrome. Plasma metanephrines were normal. A 3 cm left adrenal mass was identified without potential ectopic sources of ACTH on imaging...
November 30, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27879325/cushing-syndrome-in-a-child-due-to-pro-opiomelanocortin-pomc-secretion-from-a-yolk-sac-tumor
#9
Evelien F Gevers, Suzanne Meredith, Pratik Shah, John Torpiano, Catherine Peters, Neil J Sebire, Olga Slater, Anne White, Mehul T Dattani
CONTEXT: Pituitary microadenomas and adrenal tumours are the most common causes for endogenous Cushing syndrome (CS) in children. CASE DESCRIPTION: We describe a two-year old girl with Cushing syndrome due to ectopic pro-opiomelanocortin (POMC) production from an abdominal yolk sac tumor. Cortisol concentrations were elevated but adrenocorticotropic hormone (ACTH) concentrations were equivocal. The use of antibodies specifically detecting ACTH precursors revealed that plasma ACTH precursors were elevated...
February 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27855236/vandetanib-induces-a-marked-anti-tumor-effect-and-amelioration-of-ectopic-cushing-s-syndrome-in-a-medullary-thyroid-carcinoma-patient
#10
Hashem Bseiso, Naama Lev-Cohain, David J Gross, Simona Grozinsky-Glasberg
: A 55-year-old woman diagnosed with sporadic MTC underwent total thyroidectomy 20 years ago. After the first surgery, elevated calcitonin levels in parallel with local disease persistence were noted and therefore she underwent repeated neck dissections. During follow-up, multiple foci of metastatic disease were noted in the neck and mediastinal lymph nodes, lungs and bones; however, the disease had an indolent course for a number of years, in parallel with a calcitonin doubling time of more than two years and without significant symptoms...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27746436/metastatic-pancreatic-neuroendocrine-tumor-that-progressed-to-ectopic-adrenocorticotropic-hormone-acth-syndrome-with-growth-hormone-releasing-hormone-ghrh-production
#11
Rie Tadokoro, Shotaro Sato, Fumiko Otsuka, Makoto Ueno, Shinichi Ohkawa, Hideki Katakami, Matsuo Taniyama, Shoichiro Nagasaka
The patient was a 61-year-old woman who had a well-differentiated pancreatic neuroendocrine tumor (PNET) with lymph node metastasis. After 15 months of octreotide treatment, glucose control deteriorated and pigmentation of the tongue and moon face developed, leading to the diagnosis of ectopic adrenocorticotropic hormone (ACTH) syndrome. An abnormal secretion of growth hormone (GH) was identified, and the plasma growth hormone-releasing hormone (GHRH) level was elevated. A tumor biopsy specimen positively immunostained for ACTH and GHRH...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27725464/ectopic-adrenocorticotropic-hormone-syndrome-caused-by-neuroendocrine-carcinoma-of-the-colon
#12
Kazuyo Fujimoto, Takatoshi Nakashima, Kazunari Sasaki, Kenichi Hayashi, Masao Hanafusa, Shiei Yoshida, Satoshi Myojo, Shun-Ichi Yoshida, Shigeaki Sawai, Nobuya Sano
A 48-year-old woman with a history of autoimmune hemolytic anemia and taking long-term corticosteroid therapy presented with a 3-month history of general fatigue, abdominal distension, and pigmentation. A computed tomography scan of the abdomen showed a tumor in the sigmoid colon and multiple metastatic nodules in the liver. A colonoscopy revealed an obstructing mass with the presence of an irregular ulcer in the sigmoid colon. Following biopsy and histopathological analysis, the patient was diagnosed with neuroendocrine carcinoma (NEC) of the colon...
2016: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/27699708/frequency-of-cushing-s-syndrome-due-to-acth-secreting-adrenal-medullary-lesions-a-retrospective-study-over-10-years-from-a-single-center
#13
Henrik Falhammar, Jan Calissendorff, Charlotte Höybye
Cushing's syndrome due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions has occasionally been described. We retrospectively reviewed all 164 cases of Cushing's syndrome and 77 cases of pheochromocytomas during 10 years. Of all cases with Cushing's syndrome, only two cases (1.2 %) were due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions (one case of pheochromocytoma and one case of adrenal medullary hyperplasia). Of all pheochromocytomas only the above-mentioned case (1...
October 3, 2016: Endocrine
https://www.readbyqxmd.com/read/27695589/ectopic-cushing-s-syndrome-due-to-retroperitoneal-acth-producing-paragangliomas
#14
Fan Chen, Xiangyu Wang, Yang Wang, Hui Meng, Xinguo Hou, Yaofeng Zhu, Wei Gao, Xuewen Jiang, Shouzhen Chen, Zhaocun Zhang, Zhichuan Zou, Tianyi He, Yue Yang, Kejia Zhu, Yong Wang, Yaxiao Liu, Jianfeng Cui, Benkang Shi, Gang Yin
Extra-adrenal pheochromocytomas, or paragangliomas, are rare tumours that derive from extra-adrenal chromaffin cells. Cushing's syndrome (CS) caused by paragangliomas is extremely rare. We report a 53-year-old man with hypertension, diabetes, and symptoms of hypokalemia. Computer tomography (CT) revealed two retroperitoneal masses and bilateral adrenal hyperplasia. Together with the laboratory examinations, ectopic CS caused by multiple paragangliomas was highly suspected. The patient underwent resections of retroperitoneal tumours, left kidney, and left adrenal; postoperative histopathology confirmed two paragangliomas that were both positively stained for adrenocorticotropic hormone (ACTH)...
September 2016: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/27562041/-etiology-analysis-of-522-hospitalized-cases-with-cushing-syndrome
#15
L L Li, J T Dou, G Q Yang, W J Gu, Z H Lü, Y M Mu
OBJECTIVE: To summarize the characteristics of the etiology distribution of Cushing syndrome (CS) patients in Chinese PLA general hospital during the past 20 years. METHODS: All data of CS patients admitted to Chinese PLA General Hospital between 1995 and 2014 were collected. The sex ratio of patients, visiting age, final diagnosis were retrospectively analyzed. RESULTS: From January 1995 to December 2014, 522 patients with CS were admitted, of which, 134 (25...
August 16, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27547198/acth-antagonists
#16
REVIEW
Adrian John Clark, Rachel Forfar, Mashal Hussain, Jeff Jerman, Ed McIver, Debra Taylor, Li Chan
Adrenocorticotropin (ACTH) acts via a highly selective receptor that is a member of the melanocortin receptor subfamily of type 1 G protein-coupled receptors. The ACTH receptor, also known as the melanocortin 2 receptor (MC2R), is unusual in that it is absolutely dependent on a small accessory protein, melanocortin receptor accessory protein (MRAP) for cell surface expression and function. ACTH is the only known naturally occurring agonist for this receptor. This lack of redundancy and high degree of ligand specificity suggests that antagonism of this receptor could provide a useful therapeutic aid and a potential investigational tool...
2016: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/27523914/ectopic-adrenocortical-carcinoma-located-in-the-ovary
#17
Farida Chentli, Nadia Terki, Said Azzoug
AIM: Ovarian corticosteroid-producing tumors are exquisitely rare. Our aim was to describe the first case observed in our practice. CASE HISTORY: A 34-year-old female was referred for Cushing's syndrome (CS) occurring in the postpartum period. Clinical examination showed severe CS with diabetes mellitus, hypertension, and a large mass in the right lower abdomen. Biochemistry demonstrated corticotropin (ACTH)-independent CS (cortisol=1900ng/mL (n=50-250), ACTH<10pg/mL (n=20-46)) with estradiol and testosterone overproduction...
October 2016: European Journal of Endocrinology
https://www.readbyqxmd.com/read/27489549/role-of-acth-in-the-interactive-paracrine-regulation-of-adrenal-steroid-secretion-in-physiological-and-pathophysiological-conditions
#18
REVIEW
Hervé Lefebvre, Michaël Thomas, Céline Duparc, Jérôme Bertherat, Estelle Louiset
In the normal human adrenal gland, steroid secretion is regulated by a complex network of autocrine/paracrine interactions involving bioactive signals released by endothelial cells, nerve terminals, chromaffin cells, immunocompetent cells, and adrenocortical cells themselves. ACTH can be locally produced by medullary chromaffin cells and is, therefore, a major mediator of the corticomedullary functional interplay. Plasma ACTH also triggers the release of angiogenic and vasoactive agents from adrenocortical cells and adrenal mast cells and, thus, indirectly regulates steroid production through modulation of the adrenal blood flow...
2016: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/27464539/-comparison-of-efficacy-between-the-serum-cortisol-and-24-hour-urine-free-cortisol-in-combined-dexamethasone-suppression-test-in-the-diagnosis-of-cushing-syndrome
#19
L Lu, J H Chen, H J Zhu, A L Song, M Li, S Chen, H Pan, F Y Gong, R Z Wang, B Xing, Y Yao, M Feng, Z L Lu
OBJECTIVE: To compare the sensitivity and specificity between the 24 hour urine free cortisol (24 h UFC) and serum cortisol in dexamethasone suppression test (DST) in the diagnosis of Cushing syndrome (CS). METHODS: Combined low dose DST (LDDST) and high dose DST (HDDST) were carried out in 67 cases of CS with surgically confirmed cases in recent 3 years(from January 2011 to November 2015). The serum cortisol and 24 h UFC were collected simultaneously for each subject and the sensitivity and specificity of serum cortisol and 24 h UFC were compared...
July 19, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/27454103/therapeutic-compounds-for-cushing-s-syndrome-a-patent-review-2012-2016
#20
Li Ma, Lina Yin, Qingzhong Hu
Endogenous Cushing's syndrome (CS) is a set of disorders caused by chronic exposure to excess glucocorticoids induced by neuroendocrine tumors in pituitary, adrenals, and infrequently other sites (ectopic ACTH syndrome). Due to various comorbidities, CS patients exhibit higher risks of cardiovascular diseases and thus increased mortality. Pharmaceutical therapy is an important constituent of treatment regimen. Areas covered: Patents published since 2012 are reviewed, which claim therapeutic compounds interfering with ACTH secretion and down-stream signal transduction, inhibiting cortisol biosynthesis and antagonizing glucocorticoid receptors...
November 2016: Expert Opinion on Therapeutic Patents
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