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Pheochromocytoma

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https://www.readbyqxmd.com/read/29339836/the-role-of-metabolic-enzymes-in-mesenchymal-tumors-and-tumor-syndromes-genetics-pathology-and-molecular-mechanisms
#1
REVIEW
Inga-Marie Schaefer, Jason L Hornick, Judith V M G Bovée
The discovery of mutations in genes encoding the metabolic enzymes isocitrate dehydrogenase (IDH), succinate dehydrogenase (SDH), and fumarate hydratase (FH) has expanded our understanding not only of altered metabolic pathways but also epigenetic dysregulation in cancer. IDH1/2 mutations occur in enchondromas and chondrosarcomas in patients with the non-hereditary enchondromatosis syndromes Ollier disease and Maffucci syndrome and in sporadic tumors. IDH1/2 mutations result in excess production of the oncometabolite (D)-2-hydroxyglutarate...
January 16, 2018: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/29335064/real-time-assessment-of-cytosolic-mitochondrial-and-nuclear-calcium-levels-change-in-rat-pheochromocytoma-cells-during-pulsed-microwave-exposure-using-a-genetically-encoded-calcium-indicator
#2
Shao Hua Hu, Hui Wang, Li Lu, Hong Mei Zhou, Chang Zhen Wang, Ya Bing Gao, Ji Dong, Rui Yun Peng
Little information is available about the effects of exposure to pulsed microwaves on neuronal Ca2+ signaling under non-thermal conditions. In this study, rat pheochromocytoma (PC12) cells were exposed to pulsed microwaves for 6 min at a specific absorption rate (SAR) of 4 W/kg to assess possible real-time effects. During microwave exposure, free calcium dynamics in the cytosol, mitochondria, and nucleus of cells were monitored by time-lapse microfluorimetry using a genetically encoded calcium indicator (ratiometric-pericam, ratiometric-pericam-mt, and ratiometric-pericam-nu)...
December 2017: Biomedical and Environmental Sciences: BES
https://www.readbyqxmd.com/read/29334561/preoperative-18f-fdg-pet-ct-in-pheochromocytomas-and-paragangliomas-allows-for-precision-surgery
#3
Pavel Nockel, Mustapha El Lakis, Apostolos Gaitanidis, Roxanne Merkel, Dhaval Patel, Naris Nilubol, Tamara Prodanov, Karel Pacak, Electron Kebebew
BACKGROUND: Fluorodeoxyglucose (F-FDG) positron emission tomography/computed tomography (PET/CT) imaging is recommended in patients with metastatic pheochromocytoma (PC) and paraganglioma (PGL). There are no data on whether routine preoperative F-FDG PET/CT in all patients with PC/PGL impacts surgical management. OBJECTIVE: The aim of this study was to determine whether routine preoperative F-FDG PET/CT imaging affects the surgical management of patients with PC/PGLs...
January 12, 2018: Annals of Surgery
https://www.readbyqxmd.com/read/29327532/adrenal-oncocytoma-a-rare-functional-tumor-presenting-as-cushing-syndrome
#4
Hozefa Runderawala, Samrat Shah, Abizer Manked
Adrenal oncocytoma is very rare pathological variant of adrenal neoplasm. These are usually large and non-functional; however, rarely functional adrenal oncocytomas are also presented as Cushing's syndrome and pheochromocytoma. We report a case of adrenal oncocytoma in 38 year old female presented with symptoms of Cushing Syndrome.
December 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29325527/single-center-experience-with-laparoscopic-adrenalectomy-on-a-large-clinical-series
#5
Giovanni Conzo, Claudio Gambardella, Giancarlo Candela, Alessandro Sanguinetti, Andrea Polistena, Guglielmo Clarizia, Renato Patrone, Francesco Di Capua, Chiara Offi, Mario Musella, Sergio Iorio, Giseppe Bellastella, Daniela Pasquali, Annamaria De Bellis, Antonio Sinisi, Nicola Avenia
BACKGROUND: Laparoscopic adrenalectomy is considered the gold standard technique for the treatment of benign small and medium size adrenal masses (<6 cm), due to low morbidity rate, short hospitalization and patient rapid recovery. The aim of our study is to analyse the feasibility and efficiency of this surgical approach in a broad spectrum of adrenal gland pathologies. METHODS: Pre-operative, intra-operative and post-operative data from 126 patients undergone laparoscopic adrenalectomy between January 2003 and December 2015 were retrospectively collected and reviewed...
January 11, 2018: BMC Surgery
https://www.readbyqxmd.com/read/29317965/perioperative-anesthetic-management-of-a-case-of-rare-ectopic-pheochromocytoma
#6
Aihua Jiang, Haiqiao Zhang, Xiaofei Liu, Huishan Zhao
We report an unusual case of pheochromacytoma and investigate the perioperative anesthetic management methods for giant abdominal aortic pheochromocytoma. We analyzed and summarized the recent clinical anesthetic management and experience in the hospital for a giant abdominal aortic pheochromocytoma, with the size of 20 × 14 × 5 cm. Under general anesthesia combined with TAPB, the resection of the giant abdominal aorta pheochromocytoma was successfully completed, and the patient was cured and discharged. Well planned and careful management resulted in patient with stable blood pressure, blood volume and cardiac functions, free from complications...
December 2017: World Journal of Oncology
https://www.readbyqxmd.com/read/29315604/trends-of-genetic-screening-in-patients-with-pheochromocytoma-and-paraganglioma-15-year-experience-in-a-high-volume-tertiary-referral-center
#7
Ammar Asban, Wouter P Kluijfhout, Frederick T Drake, Toni Beninato, Elizabeth Wang, Kate Chomsky-Higgins, Wen T Shen, Jessica E Gosnell, Insoo Suh, Quan-Yang Duh
BACKGROUND AND OBJECTIVES: Genetic testing for pheochromocytoma and paraganglioma allows for early detection of hereditary syndromes and enables close follow-up of high-risk patient. We investigated the trends in genetic testing among patients at a high-volume referral center and evaluated the prevalence of pheochromocytomas and paragangliomas. METHODS: We reviewed the charts of 129 patients who underwent adrenalectomy for pheochromocytoma and paraganglioma between January 2000 and July 2015...
January 8, 2018: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29305799/prevention-of-serious-skeletal-related-events-by-interventional-radiology-techniques-in-patients-with-malignant-paraganglioma-and-pheochromocytoma
#8
Guillaume Gravel, Sophie Leboulleux, Lambros Tselikas, Flora Fassio, Mohamed Berraf, Amandine Berdelou, Bakar Ba, Segolene Hescot, Julien Hadoux, Martin Schlumberger, Abir Al Ghuzlan, France Nguyen, Mathieu Faron, Thierry de Baere, Eric Baudin, Frederic Deschamps
PURPOSE: Bone metastases (BM) and skeletal-related events (SRE) are frequent in patients with malignant pheochromocytoma and paraganglioma (PPM) and the best modality of prevention unknown. The role of interventional radiology (IR) techniques for the prevention of SRE in the multidisciplinary management of malignant PPM has not been evaluated in that setting. METHODS: Single referral center retrospective review of all patients with malignant PPM with BM from 2000 to 2016...
January 5, 2018: Endocrine
https://www.readbyqxmd.com/read/29301234/the-effect-of-coatings-and-nerve-growth-factor-on-attachment-and-differentiation-of-pheochromocytoma-cells
#9
Anna Orlowska, Pallale Tharushi Perera, Mohammad Al Kobaisi, Andre Dias, Huu Khuong Duy Nguyen, Shahram Ghanaati, Vladimir Baulin, Russell J Crawford, Elena P Ivanova
Cellular attachment plays a vital role in the differentiation of pheochromocytoma (PC12) cells. PC12 cells are noradrenergic clonal cells isolated from the adrenal medulla of Rattus norvegicus and studied extensively as they have the ability to differentiate into sympathetic neuron-like cells. The effect of several experimental parameters including (i) the concentration of nerve growth factor (NGF); (ii) substratum coatings, such as poly-L-lysine (PLL), fibronectin (Fn), and laminin (Lam); and (iii) double coatings composed of PLL/Lam and PLL/Fn on the differentiation process of PC12 cells were studied...
December 31, 2017: Materials
https://www.readbyqxmd.com/read/29288372/robotic-assisted-laparoscopic-surgery-for-pediatric-tumors-a-bicenter-experience
#10
P Meignan, Q Ballouhey, J Lejeune, K Braik, B Longis, A R Cook, H Lardy, L Fourcade, Aurélien Binet
Mini-invasive surgery is more and more integrated in pediatric surgery. The robotic-assisted surgery brought new advantages from which the patient and the surgeon could benefit compared to laparoscopy. Its use in oncological surgery is still controversial. 12 robotic-assisted tumor resections with the da Vinci Surgical Robot (Intuitive Surgical, Sunnyvale, CA) were attempted in 11 children (mean age 7.65 years; age range 0.75-16.75 years; mean weight 30.3 kg; weight range 8.6-62 kg) in two centers. Mean total operative time was 145 min (range 72-263 min)...
December 29, 2017: Journal of Robotic Surgery
https://www.readbyqxmd.com/read/29284365/echocardiographic-guidance-for-surgical-excision-of-the-intracardiac-component-of-a-pheochromocytoma
#11
Dinesh J Kurian, Angela X Li, Srikar Rao, Agathoklis Konstantinidis, Sanziana A Roman, Miklos D Kertai
Pheochromocytomas are rare neuroendocrine tumors, with published incidence of 2 to 8 cases per million patients per year. The extension of these tumors into the vena cava and right atrium is rarely seen. Transesophageal echocardiography may be invaluable to delineate tumor extent and characteristics, which in turn may provide a useful tool to guide intraoperative surgical approach to these uncommon masses. In the case presented in this article, we describe the role of transesophageal echocardiography in guiding a safe and complete, excision of an invasive pheochromocytoma without embolization of tumor components...
December 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29283372/neuroprotective-effects-and-mechanisms-of-curcumin-cu-ii-and-zn-ii-complexes-systems-and-their-pharmacological-implications
#12
Fa-Shun Yan, Jian-Long Sun, Wen-Hai Xie, Liang Shen, Hong-Fang Ji
Alzheimer's disease (AD) is the main form of dementia and has a steadily increasing prevalence. As both oxidative stress and metal homeostasis are involved in the pathogenesis of AD, it would be interesting to develop a dual function agent, targeting the two factors. Curcumin, a natural compound isolated from the rhizome of Curcuma longa, is an antioxidant and can also chelate metal ions. Whether the complexes of curcumin with metal ions possess neuroprotective effects has not been evaluated. Therefore, the present study was designed to investigate the protective effects of the complexes of curcumin with Cu(II) or Zn(II) on hydrogen peroxide (H₂O₂)-induced injury and the underlying molecular mechanisms...
December 28, 2017: Nutrients
https://www.readbyqxmd.com/read/29282712/homozygous-tmem127-mutations-in-two-patients-with-bilateral-pheochromocytomas
#13
K Eijkelenkamp, M J W Olderode-Berends, R B van der Luijt, M Robledo, M van Dooren, R A Feelders, J de Vries, M N Kerstens, T P Links, A N A van der Horst-Schrivers
Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors that are hereditary in up to 50% of patients. The gene encoding transmembrane-protein-127 (TMEM127) is one of the PCC/PGL susceptibility genes with an autosomal dominant inheritance pattern. Here we report two patients with bilateral PCC who both harbored a homozygous TMEM127-mutation. In a 31-year old mentally retarded patient the homozygous c.410-2A>G mutation was discovered during an update of DNA-analysis. A 26-year old mentally retarded patient was found to have a homozygous c...
December 28, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29282558/synchronous-bilateral-pheochromocytomas-and-paraganglioma-with-novel-germline-mutation-in-max-a-case-report
#14
Masahiro Shibata, Takahiro Inaishi, Noriyuki Miyajima, Yayoi Adachi, Yuko Takano, Kenichi Nakanishi, Dai Takeuchi, Sumiyo Noda, Yuichi Aita, Kazuhiro Takekoshi, Yasuhiro Kodera, Toyone Kikumori
BACKGROUND: Recent advance of genetic testing has contributed to the diagnosis of hereditary pheochromocytoma and paraganglioma (PPGL). The clinical characteristics of hereditary PPGL are varying among the types of mutational genes. It is still difficult to specify the pathognomonic symptoms in the case of rare genetic mutations. Here, we report the case of synchronous bilateral pheochromocytomas and paraganglioma with novel MYC associated factor X (MAX) gene mutation. CASE PRESENTATION: A 24-year-old female had hyperhidrosis and hypertension...
December 28, 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/29279858/unsupervised-network-analysis-of-the-plastic-supraoptic-nucleus-transcriptome-predicts-caprin2-regulatory-interactions
#15
Su-Yi Loh, Thomas Jahans-Price, Michael P Greenwood, Mingkwan Greenwood, See-Ziau Hoe, Agnieszka Konopacka, Colin Campbell, David Murphy, Charles C T Hindmarch
The supraoptic nucleus (SON) is a group of neurons in the hypothalamus responsible for the synthesis and secretion of the peptide hormones vasopressin and oxytocin. Following physiological cues, such as dehydration, salt-loading and lactation, the SON undergoes a function related plasticity that we have previously described in the rat at the transcriptome level. Using the unsupervised graphical lasso (Glasso) algorithm, we reconstructed a putative network from 500 plastic SON genes in which genes are the nodes and the edges are the inferred interactions...
November 2017: ENeuro
https://www.readbyqxmd.com/read/29279477/decreased-serum-adiponectin-level-during-catecholamine-crisis-in-an-obese-patient-with-pheochromocytoma
#16
Yukiyoshi Okauchi, Chisaki Ishibashi, Kunihiko Shu, Shiro Adachi, Ikuo Mineo
We herein report the case of a 37-year-old man with both pheochromocytoma and visceral fat accumulation and describe the sequential changes in his adiponectin levels throughout the clinical course from catecholamine crisis until the follow-up for adrenalectomy. His adiponectin level decreased during catecholamine crisis and increased after adrenalectomy. However, his adiponectin level decreased again at two years postoperatively when his visceral fat area greatly increased. This case suggests that catecholamines and visceral fat volume may affect adiponectin metabolism in subjects with pheochromocytoma, which may precipitate cardiovascular complications in this endocrine disease...
December 27, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29276876/a-pheochromocytoma-case-diagnosed-as-adrenal-incidentaloma
#17
Doğuş Vurallı, Nurgün Kandemir, Graeme Clark, Diclehan Orhan, Ayfer Alikaşifoğlu, Nazlı Gönç, Saniye Ekinci, Alev Özön
Vurallı D, Kandemir N, Clark G, Orhan D, Alikaşifoğlu A, Gönç N, Ekinci S, Özön A. A pheochromocytoma case diagnosed as adrenal incidentaloma. Turk J Pediatr 2017; 59: 200-206. There are two problems that needs to be addressed in cases of an adrenal incidentaloma. The first is to decide whether the adrenal mass is benign or malignant, and the second is to determine whether the mass is hormonally active or not. A 17-year-old male was admitted with the complaint of progressive weight gain. Abdominal ultrasonography was performed for elevation in transaminases which revealed a hypoechoic mass located in the left adrenal gland...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29276086/perioperative-management-of-pheochromocytoma-resection-in-a-patient-with-severe-aortic-stenosis
#18
Jagroop Singh Saran, Jacob Moalem, Luke Schoeniger, Konstantine Tzimas
No abstract text is available yet for this article.
November 22, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/29273559/high-specificity-of-spot-urinary-free-metanephrines-in-diagnosis-and-prognosis-of-pheochromocytomas-and-paragangliomas-by-hplc-with-electrochemical-detection
#19
Ming Zuo, Qianna Zhen, Xiaoqing Zhang, Wenbi Zou, Xiangchun Yang, Gang Tian, Zhenghu Shi, Qifu Li, Min Ding
BACKGROUND: The metanephrines (MNs) in plasma and urine were proposed as biomarkers for the diagnosis of pheochromocytomas and paragangliomas (PPGLs). However, plasma free MNs and 24h urinary fractionated MNs were not satisfactory enough in specificity for the diagnosis of PPGLs. Moreover, the collection of 24h urine was inconvenient. This work examined the diagnostic and prognostic efficiency of free MNs in spot urine for PPGLs. METHODS: We measured free MNs concentration in spot urine and plasma of 28 PPGLs patients and 155 control subjects by HPLC with electrochemical detection...
December 19, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29260252/-functional-diagnostics-in-endocrinology
#20
REVIEW
C J Auernhammer, M Reincke
When investigating many endocrinological diseases, basal laboratory parameters are not sufficient to distinguish between physiological and pathological hormone secretion. Functional diagnostics plays a decisive role in this context. Stimulation and suppression tests are used depending on whether under- or over-function needs to be diagnosed. This review article discusses selected functional tests, each of which plays an important role in current guidelines. Indications and test principles, including their performance, reliability, and limitations, are discussed...
December 19, 2017: Der Internist
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