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Pheochromocytoma

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https://www.readbyqxmd.com/read/28449504/malignant-pheochromocytoma-in-the-anterior-mediastinum-with-sternal-invasion-a-case-report
#1
Mingzhi Song, Kebin Sun, Tian Xia, Lei Zhou, Yangyang Li, Zhe Sun, Yuchi Zhang, Xianbin Zhang, Ran Sun, Bo Chen, Qingwei Tan
Pheochromocytomas are catecholamine-producing neuroendocrine tumors that usually occur in the adrenal medulla or sympathetic paraganglia. Anterior mediastinum involvement with pheochromocytoma is rare and may not present with typical symptoms. Its clinical manifestation may be unclear and a high index of suspicion is required for accurate diagnosis. We report a rare case of pheochromocytoma of the anterior mediastinum in a 51-year-old female. A painful hard mass on the sternum was the only clinical manifestation...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28438782/incidence-and-prevalence-of-multiple-endocrine-neoplasia-2b-in-denmark-a-nationwide-study
#2
Jes Sloth Mathiesen, Jens Peter Kroustrup, Peter Vestergaard, Mette Madsen, Kirstine Stochholm, Per Løgstrup Poulsen, Åse Krogh Rasmussen, Ulla Feldt-Rasmussen, Sten Schytte, Henrik Baymler Pedersen, Christoffer Holst Hahn, Jens Bentzen, Mette Gaustadnes, Torben Falck Ørntoft, Thomas van Overeem Hansen, Finn Cilius Nielsen, Kim Brixen, Anja Lisbeth Frederiksen, Christian Godballe
Extract: Multiple endocrine neoplasia 2B (MEN2B) is an autosomal dominant inherited cancer syndrome associating medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), ganglioneuromatosis of the aerodigestive tract and facial, ophthalmologic and skeletal abnormalities. MEN2B is caused by the M918T and A883F mutation of the REarranged during Transfection (RET) proto-oncogene in approximately 95% and <5% of cases, respectively. Only very few other mutations have been reported to cause MEN2B. In approximately 75% of MEN2B patients, mutations occur as de novo (Wells, et al...
April 24, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28435794/cushing-disease-in-a-patient-with-multiple-endocrine-neoplasia-type-2b
#3
Kannan Kasturi, Lucas Fernandes, Martha Quezado, Mary Eid, Leigh Marcus, Prashant Chittiboina, Mark Rappaport, Constantine A Stratakis, Brigitte Widemann, Maya Lodish
CONTEXT: Multiple endocrine neoplasia type 2B (MEN2B) is a rare autosomal-dominant cancer syndrome characterized in part by metastatic medullary thyroid cancer (MTC) and pheochromocytoma. Cushing disease is a rare cause of endogenous hypercortisolism in children. CASE DESCRIPTION: We describe a 21-year-old African-American male who was diagnosed at age 10 with an ACTH-secreting pituitary microadenoma. At age 16 he developed medullary thyroid cancer and was found to have multiple endocrine neoplasia type 2B with the characteristic M918T mutation of the RET proto-oncogene...
June 2017: J Clin Transl Endocrinol Case Rep
https://www.readbyqxmd.com/read/28432847/genotype-phenotype-correlation-in-paediatric-pheochromocytoma-and-paraganglioma-a-single-centre-experience-from-india
#4
Kranti Khadilkar, Vijaya Sarathi, Rajeev Kasaliwal, Reshma Pandit, Manjunath Goroshi, Vyankatesh Shivane, Anurag Lila, Tushar Bandgar, Nalini S Shah
BACKGROUND: Data on genotype-phenotype correlation in children is limited. Hence, we studied the prevalence of germline mutations and genotype-phenotype correlation in children with pheochromocytoma (PCC)/paraganglioma (PGL) and compared it with adult PCC/PGL cohort. METHODS: A total of 121 consecutive, unrelated, index PCC/PGL patients underwent genetic testing for five PCC/PGL susceptibility genes (RET, VHL, SDHB, SDHD and SDHC) and were evaluated for clinical diagnosis of neurofibromatosis type1 (NF1)...
April 22, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28431994/raynaud-s-phenomenon-revisiting-a-rare-sign-of-pheochromocytoma-paraganglioma
#5
Virendra Ashokrao Patil, Rajiv Kasaliwal, Manjunath R Goroshi, Anurag R Lila, Tushar Bandgar, Nalini S Shah
Paraganglioma (PGL) are rare tumors arising from extraadrenal chromaffin cells and occasionally secret catecholamines. The patient commonly presents with headache, palpitation, anxiety, diaphoresis and episodic or sustained hypertension. Rarely patient can present with Raynaud's phenomenon. We present a case of adolescent girl who presented with isolated Raynaud's phenomenon as only manifestation of metastasis of PGL three years after undergoing surgical excision of normetanephrine secreting abdominal PGL.
April 18, 2017: Urology
https://www.readbyqxmd.com/read/28431779/propranolol
#6
Abdulrahman A Al-Majed, Ahmed H H Bakheit, Hatem A Abdel Aziz, Fahad M Alajmi, Haitham AlRabiah
Propranolol is a noncardioselective β-blocker. It is reported to have membrane-stabilizing properties, but it does not own intrinsic sympathomimetic activity. Propranolol hydrochloride is used to control hypertension, pheochromocytoma, myocardial infarction, cardiac arrhythmias, angina pectoris, and hypertrophic cardiomyopathy. It is also used to control symptoms of sympathetic overactivity in the management of hyperthyroidism, anxiety disorders, and tremor. Other indications cover the prophylaxis of migraine and of upper gastrointestinal bleeding in patients with portal hypertension...
2017: Profiles of Drug Substances, Excipients, and related Methodology
https://www.readbyqxmd.com/read/28431377/metabolic-regulation-and-the-anti-obesity-perspectives-of-human-brown-fat
#7
REVIEW
Camilla Scheele, Søren Nielsen
Activation of brown adipose tissue (BAT) in adult humans increase glucose and fatty acid clearance as well as resting metabolic rate, whereas a prolonged elevation of BAT activity improves insulin sensitivity. However, substantial reductions in body weight following BAT activation has not yet been shown in humans. This observation raise the possibility for feedback mechanisms in adult humans in terms of a brown fat-brain crosstalk, possibly mediated by batokines, factors produced by and secreted from brown fat...
April 11, 2017: Redox Biology
https://www.readbyqxmd.com/read/28429074/ultrastructure-of-pheochromocytoma-undescribed-morphologic-features
#8
A Beiras Fernandez, A Kornberger, M Fraga, C F Vahl, A Beiras
We examined samples of human pheochromocytoma from 11 patients aged 30-70 years including one case of malignant pheochromocytoma with a view to identifying previously unreported ultrastructural details.We identified two types of nuclear inclusions consisting of irregularly shaped singular or multiple granulofibrillar formations with a typical concentric halo, on the one hand, and accumulations of egg-shaped structures consisting of granules and microfilaments, on the other. In some of the tumor cells, membrane-covered inclusions containing parallel laminar elements arranged in a paracrystalline, periodic fashion, or mega-mitrochondriae characterized by increased electrodensity of their matrix, and fibrillary material in the spaces between the cristae were present...
April 20, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28423608/anthracyclines-suppress-pheochromocytoma-cell-characteristics-including-metastasis-through-inhibition-of-the-hypoxia-signaling-pathway
#9
Ying Pang, Chunzhang Yang, Jan Schovanek, Herui Wang, Petra Bullova, Veronika Caisova, Garima Gupta, Katherine I Wolf, Gregg L Semenza, Zhengping Zhuang, Karel Pacak
Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare, neuroendocrine tumors derived from adrenal or extra-adrenal chromaffin cells, respectively. Metastases are discovered in 3-36% of patients at the time of diagnosis. Currently, only suboptimal treatment options exist. Therefore, new therapeutic compounds targeting metastatic PHEOs/PGLs are urgently needed. Here, we investigated if anthracyclines were able to suppress the progression of metastatic PHEO. We explored their effects on experimental mouse PHEO tumor cells using in vitro and in vivo models, and demonstrated that anthracyclines, particularly idarubicin (IDA), suppressed hypoxia signaling by preventing the binding of hypoxia-inducible factor 1 and 2 (HIF-1 and HIF-2) to the hypoxia response element (HRE) sites on DNA...
April 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28421470/adrenalectomy-indications-and-options-for-treatment
#10
REVIEW
Alemanno Giovanni, Bergamini Carlo, Prosperi Paolo, Valeri Andrea
The history of adrenal surgery is longstanding. Firstly described in 1889 by Thornton, the open adrenalectomy has been for decades the only surgical approach to adrenal diseases. Nowadays, instead, several approaches to adrenal glands have been described in the literature, such as laparoscopic adrenalectomy, robotic-assisted procedure and single-incision technique. Actually, laparoscopic adrenalectomy is considered as the gold standard treatment for adrenal lesions. In fact, all functional tumors, including pheochromocytoma, are candidates for a laparoscopic approach in the absence of other contraindications...
April 18, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28414676/pheochromocytoma-of-the-organ-zuckerkandl
#11
C Lee, E Chang, J Gimenez, R McCarron
INTRODUCTION: Pheochromocytomas (PCCs);, or intra-adrenal paragangliomas (PGLs);, are neuroendocrine tumors arising within the adrenal medulla. Extra-adrenal paragangliomas may arise in the sympathetic or parasympathetic paraganglia and more rarely in other organs. One of the most common extra-adrenal sites is in the organ of Zuckerkandl, a collection of chromaffin cells near the origin of the inferior mesenteric artery or near the aortic bifurcation. The following is a case of a patient with resistant hypertension secondary to an extra-adrenal paraganglioma in the organ of Zuckerkandl...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28413794/comparative-evaluation-of-iodine-131-metaiodobenzylguanidine-and-18-fluorodeoxyglucose-positron-emission-tomography-in-assessing-neural-crest-tumors-will-they-play-a-complementary-role
#12
Soumyakanti Kundu, Purushottam Kand, Sandip Basu
BACKGROUND: 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) has established a role in the evaluation of several malignancies. However, its precise clinical role in the neural crest cell tumors continues to evolve. PURPOSE: The purpose of this study was to compare iodine-131 metaiodobenzylguanidine ((131)I-MIBG) and FDG-PET of head to head in patients with neural crest tumors both qualitatively and semiquantitatively and to determine their clinical utility in disease status evaluation and further management...
January 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28413667/paraganglioma-of-the-bladder-in-a-kidney-transplant-recipient-a-case-report
#13
Hélène Lazareth, Daniel Cohen, Viorel Vasiliu, Claire Tinel, Frank Martinez, Jean-Pierre Grünfeld, Marie-France Mamzer, Christophe Legendre, Rebecca Sberro-Soussan
Renal transplantation has been associated with a significantly increased risk of developing cancer, including bladder neoplasia, with urothelial carcinoma being the most frequent type of bladder cancer. Bladder paraganglioma, also referred to as extra-adrenal pheochromocytoma, is a rare but severe condition that may cause a severe hypertensive crisis during handling and mobilization of the tumor. We herein present the case of a 67-year-old kidney transplant recipient with a bladder polyp consistent with paraganglioma of the bladder...
April 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28412207/microrna-183-family-profiles-in-pheochromocytomas-are-related-to-clinical-parameters-and-sdhb-expression
#14
Suja Pillai, Chung Y Lo, Victor Liew, Minella Lalloz, Robert A Smith, Vinod Gopalan, Alfred King-Yin Lam
This study aims to examine the expression profiles of the miR-183 cluster (miR-96/182/183) in pheochromocytoma. Pheochromocytoma tissues were prospectively collected from 50 patients with pheochromocytoma. Expression of miR-183 cluster members and SDHB protein expression were analysed in these tissues by quantitative real-time polymerase chain reaction and immunohistochemistry respectively. The expression of miR-183 cluster members in pheochromocytomas was correlated with the clinical and pathological parameters of these patients...
April 12, 2017: Human Pathology
https://www.readbyqxmd.com/read/28409892/failure-of-mibg-scan-to-detect-metastases-in-sdhb-mutated-pediatric-metastatic-pheochromocytoma
#15
Sameer Sait, Neeta Pandit-Taskar, Shakeel Modak
(123) I-meta-iodo benzyl guanidine (MIBG) scans are considered the gold standard imaging in neuroblastoma; however, flouro deoxy glucose positron emission tomography (FDG-PET) scans have increased sensitivity in adults with pheochromocytoma/paraganglioma. We describe a pediatric patient initially considered to have localized neuroblastoma based on anatomical imaging and (123) I-MIBG scan, but subsequent investigations revealed germline succinate dehydrogenase complex iron sulfur subunit B (SDHB) mutation-associated pheochromocytoma with multiple FDG-avid skeletal metastases...
April 14, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28406733/cerebrosides-from-sea-cucumber-protect-against-oxidative-stress-in-samp8-mice-and-pc12-cells
#16
Hongxia Che, Lei Du, Peixu Cong, Suyuan Tao, Ning Ding, Fengjuan Wu, Changhu Xue, Jie Xu, Yuming Wang
Alzheimer's disease (AD) is a neurodegenerative disorder. Emerging evidence implicates β-amyloid (Aβ) plays a critical role in the progression of AD. In this study, we investigated the protective effect of cerebrosides obtained from sea cucumber against senescence-accelerated mouse prone 8 (SAMP8) mice in vivo. We also studied the effect of cerebrosides on Aβ-induced cytotoxicity on the rat pheochromocytoma cell (PC12) and the underlying molecular mechanisms. Cerebrosides ameliorated learning and memory deficits and the Aβ accumulation in demented mice, decreased the content of malondialdehyde (MDA), 8-oxo-7,8-dihydro-2'-deoxyguanosine (8-OHdG), 8-hydroxy-2'-deoxyguanosine (8-oxo-G), and nitric oxide (NO), and enhanced the superoxide dismutase (SOD) activity significantly...
April 2017: Journal of Medicinal Food
https://www.readbyqxmd.com/read/28405881/accuracy-of-recommended-sampling-and-assay-methods-for-the-determination-of-plasma-free-and-urinary-fractionated-metanephrines-in-the-diagnosis-of-pheochromocytoma-and-paraganglioma-a-systematic-review
#17
Roland Därr, Matthias Kuhn, Christoph Bode, Stefan R Bornstein, Karel Pacak, Jacques W M Lenders, Graeme Eisenhofer
PURPOSE: To determine the accuracy of biochemical tests for the diagnosis of pheochromocytoma and paraganglioma. METHODS: A search of the PubMed database was conducted for English-language articles published between October 1958 and December 2016 on the biochemical diagnosis of pheochromocytoma and paraganglioma using immunoassay methods or high-performance liquid chromatography with coulometric/electrochemical or tandem mass spectrometric detection for measurement of fractionated metanephrines in 24-h urine collections or plasma-free metanephrines obtained under seated or supine blood sampling conditions...
April 12, 2017: Endocrine
https://www.readbyqxmd.com/read/28403121/pheochromocytoma-crisis-in-icu-a-french-multicenter-cohort-study-with-emphasis-on-rescue-extracorporeal-membrane-oxygenation
#18
Bertrand Sauneuf, Nicolas Chudeau, Benoit Champigneulle, Claire Bouffard, Marion Antona, Nicolas Pichon, David Marrache, Romain Sonneville, Antoine Marchalot, Camille Welsch, Antoine Kimmoun, Bruno Bouchet, Elmi Messai, Sylvie Ricome, David Grimaldi, Jonathan Chelly, Jean-Luc Hanouz, Alain Mercat, Nicolas Terzi
OBJECTIVES: To describe the characteristics, management, and outcome of patients admitted to ICUs for pheochromocytoma crisis. DESIGN: A 16-year multicenter retrospective study. SETTING: Fifteen university and nonuniversity ICUs in France. PATIENTS: Patients admitted in ICU for pheochromocytoma crisis. INTERVENTIONS: None. MEASUREMENT AND MAIN RESULTS: We included 34 patients with a median age of 46 years (40-54 yr); 65% were males...
April 11, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28400922/pheochromocytoma-and-stress-cardiomyopathy-insight-into-pathogenesis
#19
Sahil Agrawal, Jamshid Shirani, Lohit Garg, Amitoj Singh, Santo Longo, Angelita Longo, Mark Fegley, Lauren Stone, Muhammad Razavi, Nicoleta Radoianu, Sudip Nanda
AIM: To investigate the occurrence of cardiomyopathy (CMP) in a cohort of patients with histologically proven pheochromocytoma (pheo), and to determine if catecholamine excess was causative of the left ventricular (LV) dysfunction. METHODS: A retrospective chart review spanning years 1998 through 2014 was undertaken and patients with a diagnosis of pheo confirmed with histopathologic examination were included. Presenting electrocardiograms and cardiac imaging studies were reviewed...
March 26, 2017: World Journal of Cardiology
https://www.readbyqxmd.com/read/28398370/-catecholamine-induced-myocarditis-in-pheochromocytoma
#20
Davide Muratori, Patrizia Pedrotti, Matteo Baroni, Armando Belloni, Giuseppina Quattrocchi, Angela Milazzo, Cristina Giannattasio, Alberto Roghi
Pheochromocytoma is a rare tumor, usually benign, potentially lethal in case of crisis with acute release of catecholamines. The heart is a target and the clinical presentation can mimic various cardiac conditions, thus rendering diagnosis elusive. Cardiac magnetic resonance is a valuable non-invasive diagnostic tool for the evaluation of cardiomyopathies; it allows the identification of catecholamine-induced myocarditis pattern and, in some cases, it can detect the primary tumor. The definitive treatment of pheochromocytoma is surgical, while the acute crisis may require mechanical support to circulation...
February 2017: Giornale Italiano di Cardiologia
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