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Phaeochromocytoma

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https://www.readbyqxmd.com/read/29752349/paediatric-pancreatic-neuroendocrine-tumours-in-von-hippel-lindau-disease
#1
Samuel Matthew O'Toole, Anju Sahdev, Satya Bhattacharya, Roger Feakins, Evelien F Gevers, William Drake
Extract: Pancreatic neuroendocrine tumours (pNETs) are an established feature of von Hippel-Lindau disease (VHL), occurring in up to 17% of mutation carriers (Libutti et al. 2000; Blansfield et al. 2007; Erlic et al. 2010; Igarashi et al. 2014). The natural history of VHL-pNETs is poorly characterised with metastatic disease occurring in up to 25% of affected individuals (Erlic et al. 2010). Management of this unique pNET subgroup is complicated by the potential for multifocal and metachronous disease as well as extra-pancreatic VHL-related neoplasms (e...
May 11, 2018: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29741215/role-of-dotatate-pet-ct-in-preoperative-assessment-of-phaeochromocytoma-and-paragangliomas
#2
Matti L Gild, Nikita Naik, Jeremy Hoang, Edward Hsiao, Rachel T McGrath, Mark Sywak, Stan Sidhu, Leigh Walter Delbridge, Bruce Gregory Robinson, Geoff Schembri, Roderick John Clifton-Bligh
CONTEXT: Diagnosis of paragangliomas (PGL) and phaeochromocytomas (PC) can be challenging particularly if the tumour is small. Detection of metastatic disease is important for comprehensive management of malignant PC/PGL. Somatostatin receptor imaging (SRI) agents have high sensitivity for these tumours, particularly the DOTA family of radiopharmaceuticals labelled with 68 Gallium. OBJECTIVE: To describe the utility of SRI in primary assessment (i.e. before surgery) for PC/PGL and whether measures of maximum standardized uptake (SUVmax) could be used to distinguish between adrenal adenomas and PCs...
May 9, 2018: Clinical Endocrinology
https://www.readbyqxmd.com/read/29717268/adrenomedullary-function-obesity-and-permissive-influences-of-catecholamines-on-body-mass-in-patients-with-chromaffin-cell-tumours
#3
Yaxin An, Manja Reimann, Jimmy Masjkur, Katharina Langton, Mirko Peitzsch, Timo Deutschbein, Martin Fassnacht, Natalie Rogowski-Lehmann, Felix Beuschlein, Stephanie Fliedner, Anthony Stell, Aleksander Prejbisz, Andrzej Januszewicz, Jacques Lenders, Stefan R Bornstein, Graeme Eisenhofer
BACKGROUND: Obesity-associated activation of sympathetic nervous outflow is well documented, whereas involvement of dysregulated adrenomedullary hormonal function in obesity is less clear. This study assessed relationships of sympathoadrenal function with indices of obesity and influences of circulating catecholamines on body mass. METHODS: Anthropometric and clinical data along with plasma and 24-h urine samples were collected from 590 volunteers and 1368 patients tested for phaeochromocytoma and paraganglioma (PPGL), among whom tumours were diagnosed in 210 individuals...
March 3, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/29714206/curcumin-treatment-is-associated-with-increased-expression-of-the-n-methyl-d-aspartate-receptor-nmdar-subunit-nr2a-in-a-rat-pc12-cell-line-model-of-alzheimer-s-disease-treated-with-the-acetyl-amyloid-%C3%AE-peptide-a%C3%AE-25-35
#4
Wei Qian, Haiyan Li, Ningfeng Pan, Changchun Zhang
BACKGROUND The aim of this study was to investigate the effect of curcumin treatment on the expression of the N-methyl-D-aspartate receptor (NMDAR) subunit, NR2A, in a rat PC12 cell line treated with the acetyl amyloid-β peptide, Aβ(25-35), in an in vitro model of Alzheimer's disease. MATERIAL AND METHODS PC12 cells, derived from rat phaeochromocytoma, were treated for 24 hours with increasing concentrations of curcumin (5, 10, 20, 30 µM/L) in the presence of the acetyl amyloid-β peptide, Aβ(25-35). A Cell Counting Kit-8 (CCK-8) assay was used to determine cell viability, and flow cytometry was used to measure cell apoptosis...
May 1, 2018: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29660998/the-challenge-of-improving-the-diagnostic-yield-from-metanephrine-testing-in-suspected-phaeochromocytoma-and-paraganglioma
#5
Intan Samsudin, Michael M Page, Kirsten Hoad, Paul Chubb, Melissa Gillett, Paul Glendenning, Samuel Vasikaran
Background Plasma-free metanephrines (PFM) or urinary fractionated metanephrines (UFM) are the preferred biochemical tests for the diagnosis of phaeochromocytoma and paraganglioma (PPGL). Borderline increased results should be followed up to either exclude or confirm diagnosis. Methods We extracted all PFM and UFM results reported by our laboratory over a six-month period from the laboratory information system. We categorized patients with borderline increased results according to whether follow-up testing had been performed as suggested in the initial laboratory report...
January 1, 2018: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/29622713/uncommon-presentation-rare-complication-and-previously-undescribed-oncologic-association-of-pheochromocytoma-the-great-masquerader
#6
David Lawrence, Kevan Salimian, Thorsten Leucker, Seth Martin
We describe the case of a 67-year-old man presenting with ventricular tachycardia (VT) and systolic heart failure secondary to a left adrenal phaeochromocytoma. After treatment with amiodarone, the patient's VT resolved. However, his course was complicated by femoral deep venous thrombosis secondary to an incidentally discovered dedifferentiated liposarcoma of the thigh, for which he was prescribed a course of enoxaparin. The patient was discharged with plans for adrenalectomy following achievement of sufficient preoperative heart rate and blood pressure control with alpha-adrenergic receptor blockade, but re-presented to an outside facility in haemorrhagic shock, where he ultimately expired...
April 5, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29599000/neoplasia-in-three-aye-ayes-daubentonia-madagascariensis
#7
A Rodriguez Barbon, R Cowen, C Knott, K Hughes, K Allinson, C V Williams, A Routh
Tumours diagnosed in three aged captive aye-ayes (Daubentonia madagascariensis), held in two different institutions, are described. A cerebral glioblastoma was diagnosed based on histological and immunohistochemical findings in one of the animals following initial presentation with bilateral mydriasis, absent pupillary reflex, head tilt and ataxia. A second animal was humanely destroyed due to impaired locomotion associated with spondylosis and a post-mortem diagnosis of cholangiocarcinoma was made based on histology with further confirmation with immunohistochemical labelling for cytokeratin 7...
February 2018: Journal of Comparative Pathology
https://www.readbyqxmd.com/read/29577306/clinical-safety-of-percutaneous-ultrasound-guided-fine-needle-aspiration-of-adrenal-gland-lesions-in-19-dogs
#8
J A Sumner, L Lacorcia, A M Rose, A P Woodward, J E Carter
OBJECTIVE: To evaluate the safety of fine-needle aspiration of adrenal gland lesions in dogs and to characterise the risks in a subset of patients with cytologically or histopathologically diagnosed phaeochromocytoma. MATERIALS AND METHODS: Retrospective review of medical records of dogs that underwent percutaneous ultrasound-guided fine-needle aspiration of adrenal gland lesions between August 2014 and December 2016. Nineteen dogs were identified, with three undergoing bilateral adrenal gland aspiration and one dog undergoing aspiration twice, yielding 23 cytology samples in total...
March 25, 2018: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/29519441/biodegradable-and-electroconductive-poly-3-4-ethylenedioxythiophene-carboxymethyl-chitosan-hydrogels-for-neural-tissue-engineering
#9
Chao Xu, Shui Guan, Shuping Wang, Weitao Gong, Tianqing Liu, Xuehu Ma, Changkai Sun
Electroconductive hydrogels with excellent electromechanical properties have become crucial for biomedical applications. In this study, we developed a conductive composite hydrogel via in-situ chemical polymerization based on carboxymethyl chitosan (CMCS), as a biodegradable base macromolecular network, and poly(3,4-ethylenedioxythiophene) (PEDOT), as a conductive polymer layer. The physicochemical and electrochemical properties of conductive hydrogels (PEDOT/CMCS) with different contents of PEDOT polymer were analyzed...
March 1, 2018: Materials Science & Engineering. C, Materials for Biological Applications
https://www.readbyqxmd.com/read/29498920/update-on-the-clinicopathology-of-pituitary-adenomas
#10
Chung Thong Lim, Márta Korbonits
Pituitary adenomas are the third most common central nervous system tumours and arise from the anterior pituitary within the pituitary fossa. The signs and symptoms of patients with pituitary adenomas vary from 'mass effects' caused by a large adenoma to features secondary to excess pituitary hormones produced by the functioning pituitary adenoma. Detailed histopathological assessment, based on novel classifications and the latest WHO guidelines, helps to categorise pituitary adenomas into different subtypes and identify features that, in some cases, help to predict their behaviour...
March 2, 2018: Endocrine Practice
https://www.readbyqxmd.com/read/29474096/theranostic-radiopharmaceuticals-established-agents-in-current-use
#11
James R Ballinger
Although use of the term "theranostic" is relatively recent, the concept goes back to the earliest days of nuclear medicine, with the use of radioiodine for diagnosis and therapy of benign and malignant thyroid disease being arguably the most successful molecular radiotherapy in history. A diagnostic scan with 123 I-, 124 I-, or a low activity of 131 I-iodide is followed by therapy with high activity 131 I-iodide. Similarly, adrenergic tumours such as phaeochromocytoma and neuroblastoma can be imaged with 123 I-metaiodobenzylguanidine and treated with 131 I-metaiodobenzylguanidine...
March 12, 2018: British Journal of Radiology
https://www.readbyqxmd.com/read/29460105/the-importance-of-standardisation-of-measurement-and-reference-intervals-for-detection-of-phaeochromocytoma-and-paraganglioma-ppgl
#12
REVIEW
Tomás P Griffin, Delia Bogdanet, Patrick Navin, Grace Callagy, Paula M O'Shea, Marcia Bell
A 51-year-old male presented 25 years ago with excessive sweating and haematuria. Blood pressure was labile. CT abdomen showed a large right-sided adrenal mass. Two 24-h urine collections showed elevated urinary catecholamines. Right adrenal resection was performed; a phaeochromocytoma (PC) was confirmed histologically. Two decades later, the patient represented with excessive sweating and measured variable blood pressure readings. Measurement of plasma metanephrines (PMets) showed elevated normetanephrine (NMN) [50,250 (R...
February 19, 2018: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/29396241/-delayed-recurrence-of-a-phaechromocytoma-with-metastasis-as-a-cause-of-severe-hypertension-in-a-woman-with-a-surgically-intervened-retroperitoneal-tumour
#13
C Martín Varas, P A de la Torre García, M Heras Benito, G Urzola Rodríguez, J C Castro Ramos
We present the case of a woman with a previous history of hypertension and retroperitoneal tumour. The histology was reported as a paraganglioma. There was no clinical follow-up. Years later, there was a recurrence of the phaeochromocytoma after presenting with a difficult to control hypertension, along with elevation of urinary catecholamines. The case aims to highlight the importance of performing a clinical follow-up in these patients, due to the risk of recurrence.
April 2018: Hipertensión y Riesgo Vascular
https://www.readbyqxmd.com/read/29386252/tumour-risks-and-genotype-phenotype-correlations-associated-with-germline-variants-in-succinate-dehydrogenase-subunit-genes-sdhb-sdhc-and-sdhd
#14
Katrina A Andrews, David B Ascher, Douglas Eduardo Valente Pires, Daniel R Barnes, Lindsey Vialard, Ruth T Casey, Nicola Bradshaw, Julian Adlard, Simon Aylwin, Paul Brennan, Carole Brewer, Trevor Cole, Jackie A Cook, Rosemarie Davidson, Alan Donaldson, Alan Fryer, Lynn Greenhalgh, Shirley V Hodgson, Richard Irving, Fiona Lalloo, Michelle McConachie, Vivienne P M McConnell, Patrick J Morrison, Victoria Murday, Soo-Mi Park, Helen L Simpson, Katie Snape, Susan Stewart, Susan E Tomkins, Yvonne Wallis, Louise Izatt, David Goudie, Robert S Lindsay, Colin G Perry, Emma R Woodward, Antonis C Antoniou, Eamonn R Maher
BACKGROUND: Germline pathogenic variants in SDHB/SDHC / SDHD are the most frequent causes of inherited phaeochromocytomas/paragangliomas. Insufficient information regarding penetrance and phenotypic variability hinders optimum management of mutation carriers. We estimate penetrance for symptomatic tumours and elucidate genotype-phenotype correlations in a large cohort of SDHB/SDHC / SDHD mutation carriers. METHODS: A retrospective survey of 1832 individuals referred for genetic testing due to a personal or family history of phaeochromocytoma/paraganglioma...
January 31, 2018: Journal of Medical Genetics
https://www.readbyqxmd.com/read/29357678/falsely-elevated-plasma-metanephrine-in-patients-taking-midodrine
#15
Holly Emms, George Farah, Brian Shine, Chris Boot, Barry Toole, Martin McFadden, Leo Lam, Zong-Quan Ou, Gerald Woollard, Hima Madhavaram, Campbell Kyle, Ashley B Grossman
Plasma metanephrines have become the biochemical test of choice for suspected phaeochromocytomas and paragangliomas in many institutions. We encountered two separate cases of significantly elevated plasma metanephrines in patients taking midodrine, a sympathomimetic drug used in the treatment of severe postural hypotension, in the absence of a diagnosis of phaeochromocytomas and paragangliomas. Upon stopping midodrine treatment, plasma metanephrine concentrations returned to normal in both patients. To explore the hypothesis that midodrine or its metabolite desglymidodrine might interfere with the metanephrines assay, we tested the interaction of midodrine with metanephrine assays from two different centres...
January 1, 2018: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/29341163/extent-of-surgery-for-phaeochromocytomas-in-the-genomic-era
#16
REVIEW
H M Rossitti, P Söderkvist, O Gimm
BACKGROUND: Germline mutations are present in 20-30 per cent of patients with phaeochromocytoma. For patients who develop bilateral disease, complete removal of both adrenal glands (total adrenalectomy) will result in lifelong adrenal insufficiency with an increased risk of death from adrenal crisis. Unilateral/bilateral adrenal-sparing surgery (subtotal adrenalectomy) offers preservation of cortical function and independence from steroids, but leaves the adrenal medulla in situ and thus at risk of developing new and possibly malignant disease...
January 2018: British Journal of Surgery
https://www.readbyqxmd.com/read/29332973/metabologenomics-of-phaeochromocytoma-and-paraganglioma-an-integrated-approach-for-personalised-biochemical-and-genetic-testing
#17
REVIEW
Graeme Eisenhofer, Barbara Klink, Susan Richter, Jacques Wm Lenders, Mercedes Robledo
The tremendous advances over the past two decades in both clinical genetics and biochemical testing of chromaffin cell tumours have led to new considerations about how these aspects of laboratory medicine can be integrated to improve diagnosis and management of affected patients. With germline mutations in 15 genes now identified to be responsible for over a third of all cases of phaeochromocytomas and paragangliomas, these tumours are recognised to have one of the richest hereditary backgrounds among all neoplasms...
April 2017: Clinical Biochemist. Reviews
https://www.readbyqxmd.com/read/29242944/a-laparoscopic-revelation-incidental-nodule-in-a-patient-with-bilateral-phaeochromocytoma-in-multiple-endocrine-neoplasia-type-2a
#18
Vikram Sonawane, Reyaz Singaporewalla, Nga Min En, C Rajasoorya
No abstract text is available yet for this article.
December 2017: Singapore Medical Journal
https://www.readbyqxmd.com/read/29239044/pathology-and-genetics-of-phaeochromocytoma-and-paraganglioma
#19
REVIEW
John Turchini, Veronica K Y Cheung, Arthur S Tischler, Ronald R De Krijger, Anthony J Gill
Phaeochromocytoma and paraganglioma (PHEO/PGL) are rare tumours with an estimated annual incidence of 3 per million. Advances in molecular understanding have led to the recognition that at least 30-40% arise in the setting of hereditary disease. Germline mutations in the succinate dehydrogenase genes SDHA, SDHB, SDHC, SDHD and SDHAF2 are the most prevalent of the more than 19 hereditary genetic abnormalities which have been reported. It is therefore recommended that, depending on local resources and availability, at least some degree of genetic testing should be offered to all PHEO/PGL patients, including those with clinically sporadic disease...
January 2018: Histopathology
https://www.readbyqxmd.com/read/29239032/challenges-in-surgical-pathology-of-adrenocortical-tumours
#20
REVIEW
Lori A Erickson
Adrenocortical carcinomas are rare tumours that can be diagnostically challenging. Numerous multiparametric scoring systems and diagnostic algorithms have been proposed to differentiate adrenocortical adenoma from adrenocortical carcinoma. Adrenocortical neoplasms must also be differentiated from other primary adrenal tumours, such as phaeochromocytoma and unusual primary adrenal tumours, as well as metastases to the adrenal gland. Myxoid, oncocytic and sarcomatoid variants of adrenocortical tumours must be recognized so that they are not confused with other tumours...
January 2018: Histopathology
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