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Phaeochromocytoma

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https://www.readbyqxmd.com/read/29147618/programmed-cell-death-ligands-expression-in-phaeochromocytomas-and-paragangliomas-relationship-with-the-hypoxic-response-immune-evasion-and-malignant-behavior
#1
David J Pinato, James R Black, Sebastian Trousil, Roberto E Dina, Pritesh Trivedi, Francesco A Mauri, Rohini Sharma
The hypoxic response underlies the pathogenesis and malignant behavior of PCC/PGL. Regulation of PD-1 receptor-ligand signaling, a therapeutically actionable driver of the anti-tumor immune response, is a hypoxic-driven trait across malignancies. We evaluated the prognostic role of PD ligands in association with biomarkers of hypoxia and angiogenesis in patients with PCC/PGL. Tissue microarrays sections including consecutive cases diagnosed between 1983-2011 were stained for PD-L1 and 2, hypoxia inducible factor 1a (Hif-1a), Carbonic Anhydrase IX (CaIX), Vascular Endothelial Growth Factor-A (VEGF-A)...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/29147570/management-of-an-acute-catecholamine-induced-cardiomyopathy-and-circulatory-collapse-a-multidisciplinary-approach
#2
R T Casey, B G Challis, D Pitfield, R M Mahroof, N Jamieson, C J Bhagra, A Vuylsteke, S J Pettit, K C Chatterjee
A phaeochromocytoma (PC) is a rare, catecholamine-secreting neuroendocrine tumour arising from the adrenal medulla. Presenting symptoms of this rare tumour are highly variable but life-threatening multiorgan dysfunction can occur secondary to catecholamine-induced hypertension or hypotension and subsequent cardiovascular collapse. High levels of circulating catecholamines can induce an acute stress cardiomyopathy, also known as Takotsubo cardiomyopathy. Recent studies have focused on early diagnosis and estimation of the prevalence of acute stress cardiomyopathy in patients with PC, but very little is reported about management of these complex cases...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/29102976/giant-phaeochromocytoma-presenting-with-an-acute-stroke-reappraising-phaeochromocytoma-surveillance-for-the-neurofibromatosis-type-1-phakomatosis
#3
Yingshan Lee, Leon Yuan Rui Tan, Yong Howe Ho, Melvin Khee Shing Leow
Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder associated with reduced lifespan attributed largely to malignancy and vascular causes. One of the tumours associated with NF1 is phaeochromocytoma. The phaeochromocytoma has earned the moniker, a 'great mimicker', due to its varied means of presentation. We present a patient with NF1 who was diagnosed with a giant 20 cm phaeochromocytoma after suffering from an ischaemic stroke. Current guidelines do not advocate surveillance of phaeochromocytoma in asymptomatic patients with NF1, unlike other genetic syndromes associated with phaeochromocytoma...
November 3, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29068510/characteristics-of-adrenal-incidentalomas-in-a-new-zealand-centre
#4
Z Goh, I Phillips, P J Hunt, S Soule, T J Cawood
BACKGROUND: Management of adrenal incidentalomas (AI) is becoming more conservative, based on international data showing a low incidence of functional or malignant lesions. The clinical characteristics of AI in New Zealand are unknown. Therefore, whether the AI guidelines apply to the New Zealand population is also unknown. AIMS: To investigate the clinical characteristics of patients with AI presenting to a tertiary-care centre in New Zealand. METHOD: This study prospectively evaluated consecutive patients aged 18 or older with AI, 1 cm or larger, diagnosed in Canterbury, New Zealand...
October 25, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/29066504/patient-quality-of-life-and-prognosis-in-men2
#5
Joanna Grey, Kym Winter
Multiple endocrine neoplasia type 2 (MEN2) refers to the autosomal dominant neuroendocrine tumour syndromes, MEN type 2A (MEN2A) and MEN type 2B (MEN2B). They are typified by the development of medullary thyroid carcinoma (MTC), phaeochromocytoma and parathyroid hyperplasia in MEN2A, and MTC, phaeochromocytomas, ganglioneuromatosis and skeletal abnormalities in MEN2B. The aggressiveness of MTC is variable according to genotype, and although it is still the major cause of mortality in both conditions, prognosis has improved dramatically in those diagnosed and treated at a young age thanks to predictive genetic testing...
October 24, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29027806/annals-express-clinical-evaluation-and-treatment-of-phaeochromocytoma
#6
Andrew Stuart Davison, Danielle Jones, Stuart Ruthven, Timothy Helliwell, Susannah L Shore
Phaeochromocytoma and extra adrenal paraganglioma are rare neuroendocrine tumours and have the potential to secrete adrenaline, noradrenaline and dopamine causing a myriad of clinical symptoms. Prompt diagnosis is essential for clinicians and requires a multi-disciplinary specialist approach for the clinical and laboratory investigation, diagnosis, treatment and follow-up of patients. This paper is an integrated review of the clinical and laboratory evaluation and treatment of patients suspected to have phaeochromocytoma or paraganglioma, highlighting recent developments and best practices from recent published clinical guidelines...
January 1, 2017: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/28973655/clinical-and-molecular-features-of-renal-and-pheochromocytoma-paraganglioma-tumor-association-syndrome-raptas-case-series-and-literature-review
#7
Ruth T Casey, Anne Y Warren, Jose Ezequiel Martin, Benjamin G Challis, Eleanor Rattenberry, James Whitworth, Katrina A Andrews, Thomas Roberts, Graeme R Clark, Hannah West, Philip S Smith, France M Docquier, Fay Rodger, Vicki Murray, Helen L Simpson, Yvonne Wallis, Olivier Giger, Maxine Tran, Susan Tomkins, Grant D Stewart, Soo-Mi Park, Emma R Woodward, Eamonn R Maher
Context: The co-occurrence of pheochromocytoma (PC) and renal tumors was linked to the inherited familial cancer syndrome von Hippel-Lindau (VHL) disease more than six decades ago. Subsequently, other shared genetic causes of predisposition to renal tumors and to PC, paraganglioma (PGL), or head and neck paraganglioma (HNPGL) have been described, but case series of non-VHL-related cases of renal tumor and pheochromocytoma/paraganglioma tumor association syndrome (RAPTAS) are rare. Objective: To determine the clinical and molecular features of non-VHL RAPTAS by literature review and characterization of a case series...
November 1, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28969333/safety-assessments-in-the-avoidance-of-preoperative-%C3%AE-receptor-blockade-in-phaeochromocytoma-surgery-the-pitfalls-of-a-zero-numerator
#8
H P Grocott
No abstract text is available yet for this article.
September 1, 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/28969332/author-s-reply-to-grocott-risk-of-cardiac-ischaemia-in-patients-without-%C3%AE-receptor-blockade-for-phaeochromocytoma-surgery
#9
H Groeben
No abstract text is available yet for this article.
September 1, 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/28965289/when-should-genetic-testing-be-performed-in-patients-with-neuroendocrine-tumours
#10
REVIEW
Triona O'Shea, Maralyn Druce
Neuroendocrine tumours (NETs) are a heterogenous group of tumours arising from neuroendocrine cells in several sites around the body. They include tumours of the gastroenteropancreatic system, phaeochromocytoma and paraganglioma and medullary thyroid cancer. In recent years, it has become increasingly apparent that a number of these tumours arise as a result of germline genetic mutations and are inherited in an autosomal dominant pattern. The number of genes implicated is increasing rapidly. Identifying which patients are likely to have a germline mutation enables clinicians to counsel patients adequately about their future disease risk, and allows for earlier detection of at-risk patients through family screening...
September 30, 2017: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/28924583/screening-for-primary-aldosteronism-using-the-newly-developed-ids-isys%C3%A2-automated-assay-system
#11
P M O'Shea, T P Griffin, G A Browne, N Gallagher, J J Brady, M C Dennedy, M Bell, D Wall, M Fitzgibbon
BACKGROUND: The recommended approach to screening for primary aldosteronism (PA) in at-risk populations is to determine the ratio of aldosterone concentration (serum (SAC)/plasma (PAC)) to renin measured in plasma as activity (PRA) or concentration (DRC). However, lack of assay standardisation mandates the need for method-specific decision thresholds and clinical validation in the local population. AIM: The study objective was to establish method-specific aldosterone: renin ratio (ARR) cut-offs for PA in men and women using the IDS-iSYS® assay system (IDS plc)...
April 2017: Pract Lab Med
https://www.readbyqxmd.com/read/28911298/veno-arterial-extracorporeal-membrane-oxygenation-for-severe-cardiogenic-shock-secondary-to-phaeochromocytoma-crisis
#12
R K Fung, D Arumugam, A Tjokrowidjaja, P Forrest, E L Chua, T Southwood
No abstract text is available yet for this article.
September 2017: Anaesthesia and Intensive Care
https://www.readbyqxmd.com/read/28904054/a-13-steroid-serum-panel-based-on-lc-ms-ms-use-in-detection-of-adrenocortical-carcinoma
#13
David R Taylor, Lea Ghataore, Lewis Couchman, Royce P Vincent, Ben Whitelaw, Dylan Lewis, Salvador Diaz-Cano, Gabriele Galata, Klaus-Martin Schulte, Simon Aylwin, Norman F Taylor
BACKGROUND: Adrenocortical carcinoma (ACC) is a rare malignancy, with an annual incidence of 1 or 2 cases per million. Biochemical diagnosis is challenging because up to two-thirds of the carcinomas are biochemically silent, resulting from de facto enzyme deficiencies in steroid hormone biosynthesis. Urine steroid profiling by GC-MS is an effective diagnostic test for ACC because of its capacity to detect and quantify the increased metabolites of steroid pathway synthetic intermediates...
September 13, 2017: Clinical Chemistry
https://www.readbyqxmd.com/read/28872770/hormone-secreting-adrenal-tumours-cause-severe-hypertension-and-high-rates-of-poor-pregnancy-outcome-a-ukoss-study-with-case-control-comparisons
#14
G Quartermaine, K Lambert, K Rees, P T Seed, M K Dhanjal, M Knight, D R McCance, C Williamson
OBJECTIVE: To examine the management and outcomes of adrenal tumours in pregnancy. DESIGN: A national observational, cohort study over four years using the UK Obstetric Surveillance System (UKOSS). SETTING: Consultant led obstetric units. PATIENTS: Women with phaeochromocytoma, primary aldosteronism or Cushing's syndrome diagnosed before or during pregnancy. METHODS: Clinical features of UKOSS cases were compared to those of women with adrenal tumours reported from 1985-2015...
September 5, 2017: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28856331/phaeochromocytoma-and-paraganglioma-excision-involving-the-great-vessels
#15
U Srirangalingam, K Gunganah, R Carpenter, S Bhattacharya, S J Edmondson, W M Drake
OBJECTIVE/BACKGROUND: Phaeochromocytomas and paragangliomas are vascular neuroendocrine tumours distributed between the neck and the pelvis and may be associated with catecholamine secretion. The aim of the study was to describe the complex surgical management required to excise these tumours when in close proximity to the great vessels (aorta and vena cava). METHODS: This was a retrospective case series. Patients included those undergoing surgical excision of a phaeochromocytoma or paraganglioma involving the great vessels...
2017: EJVES Short Rep
https://www.readbyqxmd.com/read/28839945/concomitant-multiple-myeloma-and-probable-phaeochromocytoma-in-a-cat
#16
Mario Cervone
CASE SUMMARY: Herein a drug-resistant IgG-lambda-type multiple myeloma associated with probable phaeochromocytoma in a cat is described. A 12-year-old cat presented with weakness, weight loss, progressive blindness and open-mouth breathing, in addition to polyuria and polydipsia of 2 months' duration. Abdominal ultrasonography revealed a left adrenal mass. Phaeochromocytoma was suspected on the basis of cytology and was associated with systemic hypertension. Biochemistry showed hyperproteinaemia...
July 2017: JFMS open reports
https://www.readbyqxmd.com/read/28808335/direct-electrochemical-measurement-of-metanephrines-in-spot-urine-samples-for-the-diagnosis-of-phaeochromocytomas
#17
Zheng-Hu Shi, Xiao-Qing Zhang, Qian-Na Zhen, Ming Zuo, Gang Tian, Yi-Fan He, Min Ding
Metanephrines (MNs) were suggested as a potential first-line biochemical index for the diagnosis of phaeochromocytomas (PHEO). In this study, we developed a simple electrochemical method for the quantitative measurement of MNs in spot urine samples. As MNs contain a hydroxyphenyl group, they could be oxidized at a certain potential to quinines, which could be further detected by the differential pulse voltammetry (DPV) method using unmodified screen-printed carbon electrode (SPCE). Meanwhile, the solid phase extraction (SPE) technique was used to eliminate the matrix effect in the samples...
August 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28746746/outcomes-of-patients-with-metastatic-phaeochromocytoma-and-paraganglioma-a-systematic-review-and-meta-analysis
#18
Oksana Hamidi, William F Young, Lucinda Gruber, John Smestad, Qi Yan, Oscar J Ponce, Larry Prokop, Mohammad Hassan Murad, Irina Bancos
OBJECTIVE: The outcomes of patients with metastatic phaeochromocytoma (PHEO) and paraganglioma (PGL) are unclear. We performed a systematic review and meta-analysis of baseline characteristics and mortality rates of patients with metastatic PHEO and PGL (PPGL). DESIGN: Ovid MEDLINE In-Process & Other Non-Indexed Citations, Ovid MEDLINE, Ovid EMBASE, Ovid Cochrane Central Register of Controlled Trials, Ovid Cochrane Database of Systematic Reviews, Scopus, Web of Science, and references of key articles were searched from inception to 2016...
July 26, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28693800/review-of-sequencing-platforms-and-their-applications-in-phaeochromocytoma-and-paragangliomas
#19
REVIEW
Suja Pillai, Vinod Gopalan, Alfred King-Yin Lam
Genetic testing is recommended for patients with phaeochromocytoma (PCC) and paraganglioma (PGL) because of their genetic heterogeneity and heritability. Due to the large number of susceptibility genes associated with PCC/PGL, next-generation sequencing (NGS) technology is ideally suited for carrying out genetic screening of these individuals. New generations of DNA sequencing technologies facilitate the development of comprehensive genetic testing in PCC/PGL at a lower cost. Whole-exome sequencing and targeted NGS are the preferred methods for screening of PCC/PGL, both having precise mutation detection methods and low costs...
August 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28680468/a-rare-cause-of-hypertension-in-pregnancy-phaeochromocytoma
#20
Sonali Shah, Lindsay Edwards, Andrew Robinson, Amy Crosthwaite, Christine Houlihan, Kathy Paizis
A 26-year-old primigravida at 35 weeks' gestation was transferred to our institution from a regional hospital for management of presumed preeclampsia. Due to the labile nature of her hypertension, further investigation was undertaken which revealed a right-sided phaeochromocytoma. Alpha blockade was commenced, and an uncomplicated elective caesarean delivery was performed at 38 weeks' gestation under spinal anaesthetic. The patient underwent an elective right laparoscopic adrenalectomy six weeks post-partum...
June 2017: Obstetric Medicine
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