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Pheochromocytoma

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https://www.readbyqxmd.com/read/27908211/pheochromocytoma-in-neurofibromatosis-type-1-during-pregnancy
#1
Pablo Remón-Ruiz, Alberto Aliaga-Verdugo, Raquel Guerrero-Vázquez
Pregnant women with neurofibromatosis type 1 (NF-1) have increased complications during gestation, including hypertensive disorders that are sometimes caused by pheochromocytoma. Pheochromocytoma is an extremely rare condition during pregnancy, and the main clinical manifestation is hypertension. If not properly treated, pheochromocytoma has high maternal and fetal mortality rates. Early recognition and adequate clinical management before delivery have led to better outcomes in the last few decades. Despite the association of NF-1 and pheochromocytoma, there are few clinical reports of these two conditions in pregnant patients...
December 2, 2016: Gynecological Endocrinology
https://www.readbyqxmd.com/read/27903577/adrenal-cushing-syndrome-with-detectable-acth-from-an-unexpected-source
#2
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/27900521/-18-f-fluorodihydroxyphenylalanine-pet-ct-in-pheochromocytoma-and-paraganglioma-relation-to-genotype-and-amino-acid-transport-system-l
#3
Chloé C Feral, Floriane S Tissot, Lionel Tosello, Nicolas Fakhry, Fréderic Sebag, Karel Pacak, David Taïeb
PURPOSE: F-FDOPA is a highly sensitive and specific radiopharmaceutical for pheochromocytoma and paraganglioma (PPGL) imaging. However, (18)F-FDOPA might be falsely negative in these tumors, especially those related to mutations in succinate dehydrogenase genes (SDHx). The aim of the present study was to evaluate the relationship between expression of L-DOPA transporters and (18)F-FDOPA PET imaging results in PPGL. METHODS: From 2007 to 2015, 175 patients with non-metastatic PPGL were evaluated by (18)F-FDOPA PET/CT for initial diagnosis/staging and follow-up...
November 29, 2016: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/27900224/perioperative-severe-hypotension-in-a-patient-with-multiple-endocrine-neoplasia-type-iib-and-bilateral-adrenalectomies-time-to-review-the-evidence-for-stress-dose-steroids
#4
Jens Tan, Acsa Zavala, Katherine B Hagan, Antoinette Van Meter, Uduak Ursula Williams, Wei Zhang, Pascal Owusu-Agyemang
Multiple endocrine neoplasia type IIb (MEN IIb) is an endocrine disorder which can manifest with tumors such as pheochromocytomas and neuromas. We present the case of a patient with MEN IIb, after bilateral adrenalectomies, on maintenance steroid replacement, who underwent a neuroma resection and developed severe hypotension. There is persistent controversy regarding the general administration of perioperative "stress dose" steroids for patients with adrenal insufficiency. While the most recent literature suggests that stress dose steroids are unnecessary for secondary adrenal insufficiency, the rarer form of primary adrenal insufficiency always requires supplemental steroids, specifically hydrocortisone, when undergoing surgical procedures...
2016: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/27899191/genetic-predisposition-to-endocrine-tumors-diagnosis-surveillance-and-challenges-in-care
#5
REVIEW
Elisabeth Joye Petr, Tobias Else
Endocrine tumor syndromes, eg, multiple endocrine neoplasia types 1 and 2, were among the first recognized hereditary predisposition syndromes to tumor development. Over time, the number of endocrine tumor syndromes has significantly expanded, eg, with the recent inclusion of hereditary paraganglioma syndromes. Associations of non-endocrine tumors with hereditary endocrine tumor syndromes and endocrine tumors with non-classical endocrine tumor syndromes have emerged. These findings have certainly expanded the scope of care, necessitating a multidisciplinary approach by a team of medical professionals and researchers, integrating shared patient decision-making at every step of surveillance, diagnosis, and treatment...
October 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/27896787/extracorporeal-membrane-oxygenation-for-pheochromocytoma-induced-cardiogenic-shock
#6
Guillaume Hekimian, Fatima Kharcha, Nicolas Bréchot, Matthieu Schmidt, Cécile Ghander, Guillaume Lebreton, Xavier Girerd, Christophe Tresallet, Jean-Louis Trouillet, Pascal Leprince, Jean Chastre, Alain Combes, Charles-Edouard Luyt
BACKGROUND: Pheochromocytoma, a rare catecholamine-producing tumor, might provoke stress-induced Takotsubo-like cardiomyopathy and severe cardiogenic shock. Because venoarterial-extracorporeal membrane oxygenation (VA-ECMO) rescue of pheochromocytoma-induced refractory cardiogenic shock has rarely been reported, we reviewed our ICU patients' presentations and outcomes. METHODS: All pheochromocytoma-induced refractory cardiogenic shock cases managed with VA-ECMO (January 2007-March 2015) were prospectively included and reviewed...
December 2016: Annals of Intensive Care
https://www.readbyqxmd.com/read/27896511/resection-of-pheochromocytoma-improves-diabetes-mellitus-in-the-majority-of-patients
#7
Toni Beninato, Wouter P Kluijfhout, Frederick Thurston Drake, James Lim, Julie S Kwon, Maggie Xiong, Wen T Shen, Jessica E Gosnell, Chienying Liu, Insoo Suh, Quan-Yang Duh
BACKGROUND: Catecholamine excess in patients with pheochromocytoma often results in impaired glucose tolerance, leading to diabetes mellitus. Little data are available on the long-term effect of surgery on diabetes. OBJECTIVE: The primary aim of this study was to determine the likelihood of diabetes cure after surgery, while secondary objectives were to determine risk factors for development of diabetes preoperatively and persistence of diabetes postoperatively...
November 28, 2016: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/27895137/successful-response-to-pegylated-interferon-alpha-in-a-recurrent-paraganglioma
#8
Thibault Bahougne, Alessio Imperiale, Gerlinde Averous, Gerard Chabrier, Nelly Burnichon, Anne-Paule Gimenez-Roqueplo, Nassim Dali-Youcef, Rossella Libé, Eric Baudin, Catherine Roy, Herve Lang, Laurence Kessler
INTRODUCTION: Early and accurate identification of recurrences in patients with aggressive paraganglioma (PGL) poses significant challenges. Moreover, the most appropriate therapeutic strategy for recurrent PGL remains to be established. RESULTS: We report the case of 52-year-old man with a history of recurrent, sporadic, left retroperitoneal PGL that relapsed twice (at eight and nine years after the first surgical intervention). At the time of the second recurrence, 2-deoxy-2-[fluorine-18]fluoro-D-glucose (FDG) positron emission tomography/computed tomography (PET/CT) demonstrated a pathologically increased tracer uptake in multiple nodular lesions (10-15 mm in size) located close to the remnant of the left renal pedicle and in the left lombar paravertebral region...
November 28, 2016: Endocrine-related Cancer
https://www.readbyqxmd.com/read/27894345/ultrasonographic-features-of-adrenal-gland-lesions-in-dogs-can-aid-in-diagnosis
#9
Elena Pagani, Massimiliano Tursi, Chiara Lorenzi, Alberto Tarducci, Barbara Bruno, Enrico Corrado Borgogno Mondino, Renato Zanatta
BACKGROUND: Ultrasonography to visualize adrenal gland lesions and evaluate incidentally discovered adrenal masses in dogs has become more reliable with advances in imaging techniques. However, correlations between sonographic and histopathological changes have been elusive. The goal of our study was to investigate which ultrasound features of adrenal gland abnormalities could aid in discriminating between benign and malignant lesions. To this end, we compared diagnosis based on ultrasound appearance and histological findings and evaluated ultrasound criteria for predicting malignancy...
November 28, 2016: BMC Veterinary Research
https://www.readbyqxmd.com/read/27882185/erratum-carcinoma-like-nonfunctional-pheochromocytoma-in-the-right-adrenal-gland-a-case-report
#10
Shingo Moriyama, Hideki Takeshita, Saori Araki, Takuo Tokairin, Makoto Kagawa, Koji Chiba, Akiko Adachi, Akira Noro
[This corrects the article DOI: 10.3892/ol.2016.4776.].
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27878303/neuroprotective-effects-of-vitexin-against-isoflurane-induced-neurotoxicity-by-targeting-the-trpv1-and-nr2b-signaling-pathways
#11
Linlin Chen, Bin Zhang, Shiqiang Shan, Xin Zhao
Vitexin is a bioactive compound extracted from hawthorn leaves, which reduces blood pressure and has anti‑inflammatory and potential anticancer effects. However, the mechanisms underlying the protective effects of vitexin against isoflurane‑induced neurotoxicity remain elusive. Therefore, the aim of the present study was to investigate these mechanisms further. Sprague Dawley rats received 1.4% isoflurane in a 100% oxygen environment for 2 h. Human PC12 pheochromocytoma neurosecretory cells were exposed to 2% isoflurane for 12 h before they were treated with 1, 10 or 100 µM vitexin for a further 24 h...
November 16, 2016: Molecular Medicine Reports
https://www.readbyqxmd.com/read/27877111/polymerizing-pyrrole-coated-poly-l-lactic-acid-co-%C3%AE%C2%B5-caprolactone-plcl-conductive-nanofibrous-conduit-combined-with-electric-stimulation-for-long-range-peripheral-nerve-regeneration
#12
Jialin Song, Binbin Sun, Shen Liu, Wei Chen, Yuanzheng Zhang, Chunyang Wang, Xiumei Mo, Junyi Che, Yuanming Ouyang, Weien Yuan, Cunyi Fan
Electrospinning and electric stimulation (ES) are both promising methods to support neuron adhesion and guide extension of neurons for nerve regeneration. Concurrently, all studies focus on either electrospinning for conduits material or ES in vitro study to accelerate nerve regeneration; few work on the combined use of these two strategies or ES in vivo study. Therefore, this study aimed to investigate the abilities of direct current ES through electrospinning conductive polymer composites composed of polypyrrole and Poly (l-lactic acid-co-ε-caprolactone) (PPY/PLCL) in peripheral nerve regeneration...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27873108/flushing-in-neuro-endocrinology
#13
REVIEW
Fady Hannah-Shmouni, Constantine A Stratakis, Christian A Koch
Cutaneous flushing is a common presenting complaint in endocrine disorders. The pathophysiology of flushing involves changes in cutaneous blood flow triggered by multiple intrinsic factors that are either related to physiology or disease. Flushing can be divided into episodic or persistent causes. Episodic flushing is mediated by the release of endogenous vasoactive mediators or medications, while persistent flushing results in a fixed facial erythema with telangiectasia and cyanosis due to slow-flowing deoxygenated blood in large cutaneous blood vessels...
November 21, 2016: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/27871582/glucagon-induced-hypertensive-emergency-a-case-report
#14
Aron Legler, Richard K Kim, Nikhil Chawla
Glucagon is well acknowledged as a sphincter of Oddi relaxant for both diagnostic and therapeutic uses in choledocholithiasis, and an empiric treatment for β-blocker overdose. Although it has been implicated in inducing cardiovascular crises in patients with asymptomatic pheochromocytoma, adverse effects in other patient populations have not been characterized. This case report describes a patient with hypertension controlled on β blockers who, after glucagon administration during an intraoperative cholangiography, experienced hypertensive emergency despite adequate pain control...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27871566/known-difficult-airway-in-a-patient-with-pheochromocytoma-a-case-report
#15
Laura Alonso Guardo, Carlos Cano Gala, Antonio Rodriguez Calvo, Adriana M Buritica Aguirre, Nadia A Dávila Arango, Elena García Fernández, Carmen Ruiz Chirosa, David Sánchez Poveda, Agustín Díaz Álvarez
: The manipulation of an airway is always a critical moment in the anesthetic management of patients with pheochromocytoma due to the high incidence of undesirable hemodynamic events in relation with the stimulus represented by the laryngoscopy. A known difficult airway in which it is necessary to carry out an orotracheal intubation while preserving spontaneous ventilation subjects the patient to a stressful situation. The objective is to obtain an acceptable level of comfort and sedation avoiding respiratory depression (Anesthesiol Clin 2015;33:233-40)...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27869719/targeted-molecular-imaging-in-adrenal-disease-an-emerging-role-for-metomidate-pet-ct
#16
REVIEW
Iosif A Mendichovszky, Andrew S Powlson, Roido Manavaki, Franklin I Aigbirhio, Heok Cheow, John R Buscombe, Mark Gurnell, Fiona J Gilbert
Adrenal lesions present a significant diagnostic burden for both radiologists and endocrinologists, especially with the increasing number of adrenal 'incidentalomas' detected on modern computed tomography (CT) or magnetic resonance imaging (MRI). A key objective is the reliable distinction of benign disease from either primary adrenal malignancy (e.g., adrenocortical carcinoma or malignant forms of pheochromocytoma/paraganglioma (PPGL)) or metastases (e.g., bronchial, renal). Benign lesions may still be associated with adverse sequelae through autonomous hormone hypersecretion (e...
November 18, 2016: Diagnostics
https://www.readbyqxmd.com/read/27867865/perioperative-hemodynamic-instability-in-patients-undergoing-laparoscopic-adrenalectomy-for-pheochromocytoma
#17
REVIEW
Magdalena Pisarska, Michał Pędziwiatr, Andrzej Budzyński
Perioperative hemodynamic instability still remains the biggest surgical and anesthetic challenge in surgery for pheochromocytoma. The aim of this review was to discuss pre-, intra- and postoperative factors that may impact on hemodynamic condition of a patient. It describes patients' preparation with appropriate medication, principles of surgical technique as well as risk factors for development of hemodynamic instability in postoperative period. Currently the gold standard in the treatment of pheochromocytoma is preoperative use of alpha-blockers and laparoscopic surgery...
October 2016: Gland Surgery
https://www.readbyqxmd.com/read/27867530/continuous-renal-replacement-therapy-for-haemodynamic-collapse-and-rhabdomyolysis-induced-by-pheochromocytoma-crisis
#18
Xi Zhou, Chuhuan Zhao, Xiafei Feng, Anweshan Samanta, Yi Nuo Lin, Jun Chen, Xiaochun Dai, Xia Hong, Qiangli Xie, Weijian Huang
Pheochromocytoma associated with pregnancy is not common. Caesarean section may induce pheochromocytoma crisis, resulting in a lethal condition. The clinical picture of pheochromocytoma crisis is extremely variable. In this report, we describe a case of severe pheochromocytoma crisis induced by caesarean section presenting with hyperpyrexia, haemodynamic collapse, muscle weakness, heart failure, and acute kidney injury. Furthermore, we report that the muscle weakness was a manifestation of rhabdomyolysis, resulting from the pheochromocytoma crisis...
December 2016: ESC Heart Failure
https://www.readbyqxmd.com/read/27867439/evaluation-of-head-and-neck-paragangliomas-by-computed-tomography-in-patients-with-pheochromocytoma-paraganglioma-syndromes
#19
Ilona Michałowska, Anna Lewczuk, Jarosław Ćwikła, Aleksander Prejbisz, Urszula Swoboda-Rydz, Mariusz I Furmanek, Małgorzata Szperl, Andrzej Januszewicz, Mariola Pęczkowska
BACKGROUND: Hereditary head and neck paragangliomas (HNP) are very often associated with pheochromocytoma-paraganglioma syndromes, which are caused by mutations in genes encoding subunits of succinate dehydrogenase (SDHx) complex. The aim of this study was to determine the frequency and location of HNP among SDHx carriers. MATERIAL/METHODS: A total of 72 patients with SDHx mutations underwent computed tomography examinations of the head and neck. HNP were present in 44 (61...
2016: Polish Journal of Radiology
https://www.readbyqxmd.com/read/27865588/pediatric-patients-with-pheochromocytoma-and-paraganglioma-should-have-routine-preoperative-genetic-testing-for-common-susceptibility-genes-in-addition-to-imaging-to-detect-extra-adrenal-and-metastatic-tumors
#20
Bruna Babic, Dhaval Patel, Rachel Aufforth, Yasmine Assadipour, Samira M Sadowski, Martha Quezado, Naris Nilubol, Tamara Prodanov, Karel Pacak, Electron Kebebew
BACKGROUND: Pediatric pheochromocytomas and paragangliomas are rare with limited data on the optimal management approach. The aim of this study was to determine the role of genetic testing and imaging to detect extra-adrenal and/or metastatic tumors in pediatric pheochromocytomas and paragangliomas. METHODS: We performed a retrospective study of 55 patients diagnosed at ≤21 years of age with pheochromocytomas and paragangliomas with analysis of data on genetic testing and multimodal imaging...
November 16, 2016: Surgery
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