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ectopic ACTH

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https://www.readbyqxmd.com/read/28343156/ectopic-acth-producing-large-cell-neuroendocrine-pancoast-tumour-presenting-as-horner-syndrome
#1
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
#2
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...
March 22, 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
#3
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: Ann Hepatobiliary Pancreat Surg
https://www.readbyqxmd.com/read/28250304/pneumocystis-pneumonia-concomitant-with-ectopic-acth-syndrome-caused-by-a-large-cell-neuroendocrine-carcinoma-of-the-thymus
#4
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
https://www.readbyqxmd.com/read/28225994/a-rare-case-of-ectopic-acth-syndrome-originating-from-malignant-renal-paraganglioma
#5
Esra Tutal, Demet Yılmazer, Taner Demirci, Evrim Cakır, Salih Sinan Gültekin, Bahadır Celep, Oya Topaloğlu, Erman Çakal
Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of a non-pituitary ACTH-secreting tumor leading to Cushing's syndrome. Only a few cases have been reported previously as causing ectopic ACTH related to paraganglioma. Herein, we present a case of Cushing's syndrome, in who was proved to be attributable to an ACTH-secreting renal malignant paraganglioma. A 40-year-old woman presented with a five-month history of newly diagnosed hypertension and diabetes, weakness, hyperpigmentation, oligomenorrhea, hirsutism, and acneiform lesions...
February 13, 2017: Archives of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28222414/urinary-steroid-metabolites-in-a-case-of-florid-ectopic-cushing-s-syndrome-and-clinical-correlations
#6
Angelos Kyriacou, Karolina M Stepien, Basil Issa
A 51-year old woman was admitted with multiple cerebral, pulmonary and intra-abdominal abscesses. The combination of apparent immunosuppression, obesity, diabetes mellitus, hypertension, hypokalaemia, osteoporotic fractures and bilateral shoulder avascular necrosis led to a clinical diagnosis of Cushing's syndrome (CS). This was biochemically confirmed as follows: midnight serum cortisol 4275 nmol/L (60-250), non-suppressed overnight dexamethasone suppression test, raised salivary cortisol 716 nmol/L (5-46) and ACTH 639 ng/L (0-46)...
October 2016: Hormones: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28202381/sphenoid-sinus-carcinoid-tumour-causing-ectopic-acth-syndrome
#7
Sanjaya Perera, Ahmad Taha
A thirty-eight year old patient presented with a gradual increase in weight and Cushingoid facies of two years duration. He also had orbital congestion, with puffy eyelids and corkscrew conjunctival vessels, associated with painful eye movements. An endocrine evaluation revealed raised cortisol and ACTH. Head imaging was performed which showed an enhancing tumour arising from the sphenoid sinus, with osseous erosion of the sphenoid sinus, extending to the nasopharynx, sellar and a small amount extending intracranially...
February 12, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28183788/prognostic-factors-in-ectopic-cushing-s-syndrome-due-to-neuroendocrine-tumors-a-multicenter-study
#8
MULTICENTER STUDY
Maria Vittoria Davi', Elisa Cosaro, Serena Piacentini, Giuseppe Reimondo, Nora Albiger, Giorgio Arnaldi, Antongiulio Faggiano, Giovanna Mantovani, Nicola Fazio, Alessandro Piovesan, Emanuela Arvat, Franco Grimaldi, Letizia Canu, Massimo Mannelli, Alberto Giacinto Ambrogio, Francesca Pecori Giraldi, Chiara Martini, Andrea Lania, Manuela Albertelli, Diego Ferone, Maria Chiara Zatelli, Davide Campana, Annamaria Colao, Carla Scaroni, Massimo Terzolo, Laura De Marinis, Sara Cingarlini, Rocco Micciolo, Giuseppe Francia
OBJECTIVE: Evidence is limited regarding outcome of patients with ectopic Cushing's syndrome (ECS) due to neuroendocrine tumors (NETs). DESIGN: We assessed the prognostic factors affecting the survival of patients with NETs and ECS. METHODS: Retrospective analysis of clinicopathological features, severity of hormonal syndrome, treatments from a large cohort of patients with NETs and ECS collected from 17 Italian centers. RESULTS: Our series included 110 patients, 58...
April 2017: European Journal of Endocrinology
https://www.readbyqxmd.com/read/28170483/diagnosis-and-treatment-of-pituitary-adenomas-a-review
#9
REVIEW
Mark E Molitch
Importance: Pituitary adenomas may hypersecrete hormones or cause mass effects. Therefore, early diagnosis and treatment are important. Observations: Prevalence of pituitary adenomas ranges from 1 in 865 adults to 1 in 2688 adults. Approximately 50% are microadenomas (<10 mm); the remainder are macroadenomas (≥10 mm). Mass effects cause headache, hypopituitarism, and visual field defects. Treatments include transsphenoidal surgery, medical therapies, and radiotherapy...
February 7, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28106911/positron-emission-tomography-computed-tomography-imaging-of-adrenocorticotropic-hormone-producing-small-cell-neuroendocrine-carcinoma-of-the-cervix
#10
Vien X Nguyen, Ba D Nguyen, Dora M Lam-Himlin
Ectopic ACTH-dependent Cushing syndrome-inducing small-cell neuroendocrine carcinoma of the cervix, undiagnosed by computed tomography and whole-body octreotide scintigraphy, was identified using F-18 FDG PET/CT imaging. This article is protected by copyright. All rights reserved.
January 20, 2017: International Journal of Gynaecology and Obstetrics
https://www.readbyqxmd.com/read/28105096/nocardiosis-in-ectopic-acth-syndrome-a-case-report-and-review-of-11-cases-from-the-literature
#11
Lichen Xu, Qiaomai Xu, Meifang Yang, Hainv Gao, Mingzhi Xu, Weihang Ma
Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) associated with nocardiosis is rare, and little information is available regarding its clinical characteristics. In this study, the case of a 35-year-old male patient who showed significant cushingoid features and had a cough with yellow phlegm for 1 month is described. Pulmonary computed tomography (CT) scanning and (18)F-fluorodeoxyglucose positron emission tomography combined with CT identified two different lesions in the mediastinum and pulmonary region, respectively...
December 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28098665/source-of-ectopic-acth-secretion-easily-identified-by-68ga-dotanoc-pet-ct
#12
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-case-of-medullary-thyroid-carcinoma-with-a-somatic-ret-mutation
#13
Anne-Cécile Paepegaey, Béatrix Cochand-Priollet, Estelle Louiset, Pierre-Olivier Sarfati, Marco Alifano, Nelly Burnichon, Marie Bienvenu-Perrard, Najiba Lahlou, Léopoldine Bricaire, Lionel Groussin
BACKGROUND: Medullary thyroid carcinomas (MTCs) complicated by ectopic Cushing's syndrome (CS) have a poor prognosis, partially due to the difficulty in controlling 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 anti-secretory action on ACTH. However, there is a lack of long-term follow-up studies. PATIENT FINDINGS: The case is reported of a 58-year-old man with MTC-related CS resistant to a combination of several anti-cortisolic drugs...
March 13, 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
#14
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...
February 2017: World Neurosurgery
https://www.readbyqxmd.com/read/27977570/hypokalemia-associated-with-a-solitary-pulmonary-nodule-a-case-report
#15
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
#16
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
#17
REVIEW
D N Paduraru, A Nica, M Carsote, A Valea
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
#18
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
#19
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
#20
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
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