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Phaeochromocytoma

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https://www.readbyqxmd.com/read/28626127/a-case-of-retroperitoneal-paraganglioma
#1
Takao Amaike, Toshihisa Tamura, Atsuhiro Koga, Kazunori Shibao, Aiichiro Higure, Keiji Hirata
We report a surgical case of retroperitoneal paraganglioma. A paraganglioma is a catecholamine-producing tumor originating in the chromaffin cells of the sympathetic ganglion. It is a kind of pheochromocytoma which occurs on the outside of the adrenal gland. The patient was a 72 year old male with a history of hypertension and a pacemaker implantation. A mass in the ventral side of the right iliopsoas muscle was detected during a routine contrasting computed tomography (CT) examination for checking his pacemaker...
2017: Journal of UOEH
https://www.readbyqxmd.com/read/28605448/efficacy-of-peptide-receptor-radionuclide-therapy-prrt-for-functional-metastatic-paraganglioma-and-phaeochromocytoma
#2
Grace Kong, Simona Grozinsky-Glasberg, Michael S Hofman, Jason Callahan, Amichay Meirovitz, Ofra Maimon, David A Pattison, David J Gross, Rodney J Hicks
Purpose: Treatment options for unresectable Paraganglioma (PGL)/Phaeochromocytoma (PCC), especially with uncontrolled secondary hypertension (HTN) are limited. Preliminary studies with PRRT suggest efficacy but data on HTN control and survival are lacking. We assessed PRRT outcomes in such patients from 2 referral centres. Methods: 20 consecutive patients (M:F=13:7; 21-77y.o.) with high somatostatin receptor (SSTR) expression treated with 177Lu-DOTA-octreotate, 9 with radiosensitising chemotherapy, were retrospectively reviewed...
June 9, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28605116/phaeochromocytoma-in-multiple-endocrine-neoplasia-type-2-ret-codon-specific-penetrance-and-changes-in-management-during-the-last-four-decades
#3
L Mucha, G Leidig-Bruckner, K Frank-Raue, Th Bruckner, M Kroiss, F Raue
OBJECTIVES: We describe phaeochromocytoma penetrance in multiple endocrine neoplasia type 2 (MEN2) according to RET protooncogene-specific mutations, and report changes in phaeochromocytoma diagnosis and management from 1968 to 2015. DESIGN: This retrospective chart review included 309 MEN2 patients from one specialised ambulatory care centre. Phaeochromocytoma patients were categorised by diagnosis date: early, 1968-1996, n = 40; and recent, 1997-2015, n = 45. RESULTS: Phaeochromocytoma was diagnosed in 85/309 patients with RET mutations in the following exons (phaeos/all carriers, %): exon 11 (56/120, 46·6%); exon 16 (7/17, 41·2%), exon 10 (14/47, 29·8%), and exon 13-15 (2/116, 1·7%)...
June 12, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28548279/significant-decreases-in-blood-propofol-concentrations-during-adrenalectomy-for-phaeochromocytoma
#4
Tatsunori Watanabe, Haruhiko Hiraoka, Takuya Araki, Daisuke Nagano, Tohru Aomori, Tomonori Nakamura, Koujirou Yamamoto, Hiroshi Baba
AIM: The kinetics of propofol are influenced by cardiac output. The aim of this study was to examine changes in blood propofol concentrations during phaeochromocytoma surgery using target-controlled infusion (TCI) anaesthesia with propofol. METHODS: This is a prospective observational study. Ten patients with phaeochromocytoma who underwent unilateral adrenalectomy were included. Cardiac output was measured using an arterial pressure-based cardiac output analysis method...
May 26, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28462638/complete-laparoscopic-excision-of-a-giant-retroperitoneal-paraganglioma
#5
Zar Jawad, A B Fajardo-Puerta, D Lefroy, J Todd, P B Lim, L R Jiao
Paragangliomas (or 'extra-adrenal phaeochromocytomas') are rare tumours arising from neural crest cells. They occur most commonly in the abdomen along the paraspinal sympathetic ganglion. The malignancy rate can be up to 35% and surgical resection is the recommended treatment. While laparoscopic excision of phaeochromocytomas is now well established, the overall number of cases of paragangliomas reported is much smaller owing to their rarity (even more so for giant paragangliomas of ≥8cm) and controversy remains over the completeness of excision...
May 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/28445897/adrenal-vein-catecholamine-levels-and-ratios-reference-intervals-derived-from-patients-with-primary-aldosteronism
#6
Candy W C Sze, Samuel Matthew O'Toole, Roger Kent Tirador, Scott A Akker, Matthew Matson, Leslie Perry, Maralyn Rose Druce, Tanja Dekkers, Jaap Deinum, Jacques W M Lenders, Graeme Eisenhofer, William Martyn Drake
Phaeochromocytoma localisation is generally reliably achieved with modern imaging techniques, particularly in sporadic cases. On occasion, however, there can be diagnostic doubt due to the presence of bilateral adrenal abnormalities, particularly in patients with mutations in genes predisposing them to the development of multiple phaeochromocytomas. In such cases, surgical intervention is ideally limited to large or functional lesions due to the long-term consequences associated with hypoadrenalism. Adrenal venous sampling (AVS) for catecholamines has been used in this situation to guide surgery, although there are few data available to support diagnostic thresholds...
April 26, 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/28429830/establishment-and-evaluation-of-a-novel-biomarker-based-nomogram-for-malignant-phaeochromocytomas-and-paragangliomas
#7
Xu Zhong, Lei Ye, TingWei Su, Jing Xie, Weiwei Zhou, Yiran Jiang, Lei Jiang, Guang Ning, Weiqing Wang
OBJECTIVE: No single histological or molecular marker is diagnostic for malignant phaeochromocytomas and paragangliomas (PPGLs). This study aimed to establish and evaluate a prognostic nomogram to improve the prediction of metastatic probability in individual PPGL patients. METHODS: Three hundred and forty-seven consecutive PPGL patients from January 2002 through December 2014 were randomly divided into a training set (n=208) and a validation set (n=139). A multivariate logistic regression analysis of selected prognostic features was performed, and a nomogram to predict metastasis was constructed...
April 21, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28385489/developmental-neurotoxicity-of-different-pesticides-in-pc-12-cells-in-vitro
#8
COMPARATIVE STUDY
Verena Christen, Manuel Rusconi, Pierre Crettaz, Karl Fent
The detection of developmental neurotoxicity (DNT) of chemicals has high relevance for protection of human health. However, DNT of many pesticides is only little known. Furthermore, validated in vitro systems for assessment of DNT are not well established. Here we employed the rat phaeochromocytoma cell line PC-12 to evaluate DNT of 18 frequently used pesticides of different classes, including neonicotinoids, pyrethroids, organophosphates, organochlorines, as well as quaternary ammonium compounds, the organic compound used in pesticides, piperonyl butoxide, as well as the insect repellent diethyltoluamide (DEET)...
June 15, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/28369925/cousins-not-twins-intratumoural-and-intertumoural-heterogeneity-in-syndromic-neuroendocrine-tumours
#9
Aidan Flynn, Trisha Dwight, Diana Benn, Siddhartha Deb, Andrew J Colebatch, Stephen Fox, Jessica Harris, Emma L Duncan, Bruce Robinson, Annette Hogg, Jason Ellul, Henry To, Cuong Duong, Julie A Miller, Christopher Yates, Paul James, Alison Trainer, Anthony J Gill, Roderick Clifton-Bligh, Rodney J Hicks, Richard W Tothill
Hereditary endocrine neoplasias, including phaeochromocytoma/paraganglioma and medullary thyroid cancer, are caused by autosomal dominant mutations in several familial cancer genes. A common feature of these diseases is the presentation of multiple primary tumours, or multifocal disease representing independent tumour clones that have arisen from the same initiating genetic lesion, but have undergone independent clonal evolution. Such tumours provide an opportunity to discover common cooperative changes required for tumourigenesis, while controlling for the genetic background of the individual...
March 31, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28331569/an-unusual-diagnosis-for-persistent%C3%A2-diarrhoea-and-vomiting
#10
James Nott, Asad Khan, Ravi Madhotra, George MacFaul, Kamran Rostami
Identifying the etiology of chronic diarrhoea might be challenging in some patients, and before a diagnosis is made these patients may spend a substantial length of time with unresolved symptoms leading to uncertainty and anxiety that is severely impairing their life quality. A 45-year-old female was referred by her general practitioner with a 5-year history of increasingly frequent episodes of cyclical diarrhoea, vomiting, abdominal pain and intermittent palpitations. Contrast CT Abdomen/Pelvis revealed a 36x33x46 mm mass in the aorto caval region of her retro-peritoneum, just above the bifurcation...
2017: Gastroenterology and Hepatology From Bed to Bench
https://www.readbyqxmd.com/read/28261513/primary-clear-cell-microcystic-adenoma-of-the-sinonasal-cavity-pathological-or-fortuitous-association
#11
Rosalin Cooper, Hannah Markham, Jeffery Theaker, Adrian Bateman, David Bunyan, Matthew Sommerlad, Gillian Crawford, Diana Eccles
Primary clear cell microcystic adenoma of the sinonasal cavity is rare. It has previously been described only as a VHL-associated tumour. Von Hippel-Lindau (VHL) syndrome is an inherited cancer syndrome characterised by an elevated risk of neoplasia including clear cell renal cell carcinoma (ccRCC), haemangioblastoma, and phaeochromocytoma. We describe the second reported case of a primary clear cell microcystic adenoma of the sinonasal cavity. The 39-year-old patient with VHL syndrome had previously undergone resection and ablation of ccRCC...
2017: Case Reports in Pathology
https://www.readbyqxmd.com/read/28248910/high-prevalence-of-cardiac-electric-abnormalities-in-patients-with-phaeochromocytomas
#12
Elena Berra, Maria C Gonzalez, Christophe Scavée, Laurent Vroonen, Akila Bersali, Anne-Catherine Pouleur, Alexandre Persu
No abstract text is available yet for this article.
April 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28179334/potential-pitfalls-of-sdh-immunohistochemical-detection-in-paragangliomas-and-phaeochromocytomas-harbouring-germline-sdhx-gene-mutation
#13
Raffaella Santi, Elena Rapizzi, Letizia Canu, Tonino Ercolino, Gianna Baroni, Rossella Fucci, Giuseppe Costa, Massimo Mannelli, Gabriella Nesi
BACKGROUND/AIM: Germline mutations in any of the succinate dehydrogenase (SDH) genes result in destabilization of the SDH protein complex and loss of SDHB expression at immunohistochemistry. SDHA is lost together with SDHB in SDHA-mutated tumours, but its expression is retained in tumours with other SDH mutations. We investigated whether SDHA/SDHB immunohistochemistry is able to identify SDH-related tumours in a retrospective case series of phaeochromocytomas (PCCs) and paragangliomas (PGLs)...
2017: Anticancer Research
https://www.readbyqxmd.com/read/28166370/peptide-receptor-radionuclide-treatment-and-131-i-mibg-in-the-management-of-patients-with-metastatic-progressive-phaeochromocytomas-and-paragangliomas
#14
Konstantinos Nastos, Vincent T F Cheung, Christos Toumpanakis, Shaunak Navalkissoor, Anne-Marie Quigley, Martyn Caplin, Bernard Khoo
BACKGROUND AND OBJECTIVES: Radionuclide therapy has been used to treat patients with progressive/metastatic paragangliomas (PGLs) and phaeochromocytomas (PCCs). The aim of the present study is to retrospectively compare the therapeutic outcomes of these modalities in patients with progressive/metastatic PCCs and PGLs. METHODS: Patients with progressive/metastatic PGLs and PCCs that were subjected to radionuclide treatment in our department were retrieved from our department's database for the period 1998-2013...
March 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28150464/adrenomedullin-new-perspectives-of-a-potent-peptide-hormone
#15
Ria Schönauer, Sylvia Els-Heindl, Annette G Beck-Sickinger
Adrenomedullin (ADM) is a 52-amino acid multifunctional peptide, which belongs to the calcitonin gene-related peptide (CGRP) superfamily of vasoactive peptide hormones. ADM exhibits a significant vasodilatory potential and plays a key role in various regulatory mechanisms, predominantly in the cardiovascular and lymphatic system. It exerts its effects by activation of the calcitonin receptor-like receptor associated with one of the receptor activity-modifying proteins 2 or 3. ADM was first isolated from human phaeochromocytoma in 1993...
February 2, 2017: Journal of Peptide Science: An Official Publication of the European Peptide Society
https://www.readbyqxmd.com/read/28100576/perioperative-management-of-a-large-late-presenting-phaeochromocytoma
#16
Duncan Macrosson, Andrew Love
A 77-year-old man presenting to the emergency department with an acute coronary syndrome was later found to have a phaeochromocytoma. The lateness of this presentation was likely due to the protective α blocking effects of long-term terazosin therapy for his prostatism. α blockers such as terazosin are a well-recognised medical therapy in the perioperative optimisation of phaeochromocytoma because they treat the adrenergic effects of catecholamine release from the tumour such as hypertension. This patient was diagnosed with an ST elevation myocardial infarction (STEMI)...
January 18, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28100521/perioperative-%C3%AE-receptor-blockade-in-phaeochromocytoma-surgery-an-observational-case-series
#17
H Groeben, B J Nottebaum, P F Alesina, A Traut, H P Neumann, M K Walz
BACKGROUND: Mortality associated with surgery for phaeochromocytoma has dramatically decreased over the last decades. Many factors contributed to the dramatic decline of the mortality rate, and the influence of an α-receptor blockade is unclear and has never been tested in a randomized trial. We evaluated intraoperative haemodynamic conditions and the incidence of complications in patients with and without α-receptor blockade undergoing surgery for catecholamine producing tumours. METHODS: Haemodynamic conditions and perioperative complications were assessed in 110 patients with (B) and 166 without (N) α-receptor blockade...
February 2017: British Journal of Anaesthesia
https://www.readbyqxmd.com/read/28018063/endoprosthetic-reconstruction-for-metastatic-phaeochromocytoma-in-the-distal-femur-a-case-report
#18
Kanchana Pala Srikanth, Chirukuri Srinivas, Lakshmipura Gangadharaiah Gowrishankarswamy, Chikkamuniyappa Chandrasekar
Metastatic spread of malignant phaeochromocytoma is known to involve multiple organs including the axial skeleton. Its presentation as a solitary lesion in the long bones of the extremities is extremely rare. We report a unique case of solitary metastatic phaeochromocytoma presenting in the distal femur, 16 years after excision of primary abdominal tumor. A 60 year old female, operated for adrenal phaeochromocytoma 16 years back was detected to have a bone tumor in her left distal femur. Chest and abdominal CT evaluation including bone scintigraphy confirmed the lesion to be solitary...
October 2016: Journal of Clinical Orthopaedics and Trauma
https://www.readbyqxmd.com/read/27994876/disease-modifying-polymorphisms-and-c609y-mutation-of-ret-associated-with-high-penetrance-of-phaeochromocytoma-and-low-rate-of-mtc-in-men2a
#19
Rowena Speak, Jackie Cook, Barney Harrison, John Newell-Price
Mutations of the rearranged during transfection (RET) proto-oncogene, located on chromosome 10q11.2, cause multiple endocrine neoplasia type 2A (MEN2A). Patients with mutations at the codon 609 usually exhibit a high penetrance of medullary thyroid cancer (MTC), but a sufficiently low penetrance of phaeochromocytoma that screening for this latter complication has been called to question. Patients with other RET mutations are at higher risk of younger age onset phaeochromocytoma if they also possess other RET polymorphisms (L769L, S836S, G691S and S904S), but there are no similar data for patients with 609 mutations...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27987712/hyaluronic-acid-doped-poly-3-4-ethylenedioxythiophene-chitosan-gelatin-pedot-ha-cs-gel-porous-conductive-scaffold-for-nerve-regeneration
#20
Shuping Wang, Shui Guan, Zhibo Zhu, Wenfang Li, Tianqing Liu, Xuehu Ma
Conducting polymer, as a "smart" biomaterial, has been increasingly used to construct tissue engineered scaffold for nerve tissue regeneration. In this study, a novel porous conductive scaffold was prepared by incorporating conductive hyaluronic acid (HA) doped-poly(3,4-ethylenedioxythiophene) (PEDOT-HA) nanoparticles into a chitosan/gelatin (Cs/Gel) matrix. The physicochemical characteristics of Cs/Gel scaffold with 0-10wt% PEDOT-HA were analyzed and the results indicated that the incorporation of PEDOT-HA into scaffold increased the electrical and mechanical properties while decreasing the porosity and water absorption...
February 1, 2017: Materials Science & Engineering. C, Materials for Biological Applications
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