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Paraganglioma

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https://www.readbyqxmd.com/read/28234804/malignant-pheochromocytoma-paraganglioma-pathogenesis-tnm-staging-and-current-clinical-trials
#1
Alejandro Roman-Gonzalez, Camilo Jimenez
PURPOSE OF REVIEW: Pheochromocytomas and paragangliomas (PPGs) are rare neuroendocrine tumors. Over the last 15 years, substantial progress has been made toward understanding the clinical aspects and molecular origins of this disease. Nevertheless, predicting and managing malignancy remains the biggest challenge in clinical practice. The natural history of patients with malignant PPGs has not yet been described, and their prognosis varies. Currently, the diagnosis of malignant PPGs relies on the presence of metastases, by which time the disease is usually advanced...
February 23, 2017: Current Opinion in Endocrinology, Diabetes, and Obesity
https://www.readbyqxmd.com/read/28231563/hypoxia-pathway-mutations-in-pheochromocytomas-and-paragangliomas
#2
Diana Amorim-Pires, Joana Peixoto, Jorge Lima
Pheochromocytomas (PCC) and sympathetic paragangliomas (PGL) are rare neuroendocrine tumors, which derive from chromaffin cells occurring in the adrenal medulla and extra-adrenal sympathetic paraganglia. PCC and PGL are often benign, catecholamine-producing tumors, responsible for a myriad of symptoms that may be potentially hazardous to the patient. In contrast, nonsecreting parasympathetic PGL, derived from chief cells, develop mainly in the head and neck region. Although PCC/PGL are more commonly sporadic tumors, germline mutations are present in up to 40% of the patients, ranking these tumors among those with the highest degree of heritability...
February 24, 2017: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/28225994/a-rare-case-of-ectopic-acth-syndrome-originating-from-malignant-renal-paraganglioma
#3
Esra Tutal, Demet Yılmazer, Taner Demirci, Evrim Cakır, Salih Sinan Gültekin, Bahadır Celep, Oya Topaloğlu, Erman Çakal
Ectopic adrenocorticotropic hormone (ACTH) syndrome is characterized by hypercortisolism due to the hypersecretion of a non-pituitary ACTH-secreting tumor leading to Cushing's syndrome. Only a few cases have been reported previously as causing ectopic ACTH related to paraganglioma. Herein, we present a case of Cushing's syndrome, in who was proved to be attributable to an ACTH-secreting renal malignant paraganglioma. A 40-year-old woman presented with a five-month history of newly diagnosed hypertension and diabetes, weakness, hyperpigmentation, oligomenorrhea, hirsutism, and acneiform lesions...
February 13, 2017: Archives of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28224355/erratum-to-genetic-screening-in-pheochromocytoma-paraganglioma
#4
Peihua Liu, Xiongbing Zu, Longfei Liu
No abstract text is available yet for this article.
February 21, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28213067/duodenal-gangliocytic-paraganglioma-successfully-treated-by-local-surgical-excision-a-case-report
#5
Dimetrios Papaconstantinou, Nikolaos Machairas, Vasileia Damaskou, Nikolaos Zavras, Christine Kontopoulou, Anastasios Machairas
INTRODUCTION: Duodenal gangliocytic paragangliomas are rare neoplasms often arising in proximity to the major duodenal papilla of Vater. These neoplasms are considered to have a benign behavior with lymph node metastases being a rare phenomenon and distant metastatic disease even more so. Resection of the tumor is the only definitive therapy. PRESENTATION OF CASE: A 67year old male presented to a referring hospital with symptoms of fatigue and malaise. Evaluation with CT imaging revealed a 3...
January 19, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28197344/from-bad-to-worse-paraganglioma-diagnosis-during-induction-of-labor-for-coexisting-preeclampsia
#6
Sasima Dusitkasem, Blair H Herndon, Dalton Paluzzi, Joseph Kuhn, Robert H Small, John C Coffman
Pheochromocytomas and extra-adrenal paragangliomas are catecholamine-secreting tumors that rarely occur in pregnancy. The diagnosis of these tumors in pregnancy can be challenging given that many of the signs and symptoms are commonly attributed to preeclampsia or other more common diagnoses. Early diagnosis and appropriate management are essential in optimizing maternal and fetal outcomes. We report a rare case of a catecholamine-secreting tumor in which diagnosis occurring at the time labor was being induced for concomitant preeclampsia with severe features...
2017: Case Reports in Anesthesiology
https://www.readbyqxmd.com/read/28197025/current-perioperative-management-of-pheochromocytomas
#7
REVIEW
Rashmi Ramachandran, Vimi Rewari
Neuroendocrine tumors which have the potential to secrete catecholamines are either associated with sympathetic adrenal (pheochromocytoma) or nonadrenal (paraganglioma) tissue. Surgical removal of these tumors is always indicated to cure and prevent cardiovascular and other organ system complications associated with catecholamine excess. Some of these tumors have malignant potential as well. The diagnosis, localization and anatomical delineation of these tumors involve measurement of catecholamines and their metabolic end products in plasma and urine, (123)I-metaiodobenzylguanidine scintigraphy, computed tomography, and/or magnetic resonance imaging...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28196587/pheochromocytomas-and-paragangliomas-genetically-diverse-and-minimalist-all-at-once
#8
Patricia L M Dahia
Pheochromocytomas and paragangliomas are infrequent, genetically heterogeneous neuroendocrine tumors. In this issue of Cancer Cell, Fishbein et al. report novel driver genes, pathogenic mechanisms, and markers of malignancy. Notably, the new findings support the long-held view that these tumors distinctively arise from a single driver event, either inherited or acquired.
February 13, 2017: Cancer Cell
https://www.readbyqxmd.com/read/28195902/mibg-localization-of-a-subclinical-paraganglioma-of-the-bladder
#9
Ayman Mithqal, Pasha Darvishi, Patrice Rehm
Urinary bladder paragangliomas are very rare, and the majority are hormonally active. Nonfunctioning paragangliomas are even rarer and more difficult to diagnose because of absence of catecholamine secretion. We present a 27-year-old woman with an asymptomatic paraganglioma of the urinary bladder discovered incidentally in a patient with prior resection of a functional pararenal paraganglioma. Metabolic profiles and cystoscopy were nondiagnostic. We demonstrate the increased sensitivity of I-MIBG SPECT/CT compared with planar scintigraphy for detection of a nonfunctional paraganglioma of the urinary bladder, which makes it valuable in evaluation of functional and nonfunctional paragangliomas...
February 13, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28192382/endoscopic-management-of-middle-ear-paragangliomas-a-case-series
#10
Daniel E Killeen, Cameron C Wick, Jacob B Hunter, Alejandro Rivas, George B Wanna, João Flávio Nogueira, Joe Walter Kutz, Brandon Isaacson
OBJECTIVE: To investigate the efficacy and safety of endoscopic middle ear paraganglioma (glomus tympanicum) resection. STUDY DESIGN: Case series with chart review. SETTING: Multi-institutional tertiary university medical centers. PATIENTS: Adult patients with middle ear paragangliomas treated via a transcanal endoscopic approach from 1/2012 to 11/2015. INTERVENTION: All tumors were initially approached via a transcanal endoscopic technique...
March 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28192381/plasmacytoma-of-the-temporal-bone-a-great-imitator-report-of-seven-cases-and-comprehensive-review-of-the-literature
#11
Alex D Sweeney, Jacob B Hunter, S Vincent Rajkumar, John I Lane, Dragan Jevremovic, Matthew L Carlson
OBJECTIVES: To describe the clinical presentation, radiological features, and outcome of patients with plasmacytoma of the temporal bone. METHODS: Multicenter retrospective case series of all patients diagnosed with plasmacytoma of the temporal bone between 1990 and 2015. Comprehensive literature review of all previously published cases. RESULTS: A total of seven patients (average age, 57.3 yr; 57% women) met inclusion criteria. Three (43%) had a known history of multiple myeloma (MM)...
March 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28187022/carotid-body-paraganglioma-a-rare-tumor-with-serious-anesthetic-challenges
#12
Angela T Truong, Sudip Thakar, Dam-Thuy Truong
No abstract text is available yet for this article.
February 10, 2017: Anesthesiology
https://www.readbyqxmd.com/read/28187001/molecular-markers-of-paragangliomas-pheochromocytomas
#13
REVIEW
Svetlana O Zhikrivetskaya, Anastasiya V Snezhkina, Andrew R Zaretsky, Boris Y Alekseev, Anatoly V Pokrovsky, Alexander L Golovyuk, Nataliya V Melnikova, Oleg A Stepanov, Dmitry V Kalinin, Alexey A Moskalev, George S Krasnov, Alexey A Dmitriev, Anna V Kudryavtseva
Paragangliomas/pheochromocytomas comprise rare tumors that arise from the extra-adrenal paraganglia, with an incidence of about 2 to 8 per million people each year. Approximately 40% of cases are due to genetic mutations in at least one out of more than 30 causative genes. About 25-30% of pheochromocytomas/paragangliomas develop under the conditions of a hereditary tumor syndrome a third of which are caused by mutations in the VHL gene. Together, the gene mutations in this disorder have implicated multiple processes including signaling pathways, translation initiation, hypoxia regulation, protein synthesis, differentiation, survival, proliferation, and cell growth...
February 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28180050/utility-of-an-ultrasonic-aspirator-in-transcanal-endoscopic-resection-of-temporal-bone-paraganglioma
#14
Brendan P O'Connell, Jacob B Hunter, Matthew L Carlson, Brandon Isaacson, Alejandro Rivas, George B Wanna
The objective of this study was to evaluate the role of removing middle ear paragangliomas using an ultrasonic aspirator (UA) through a transcanal, exclusively endoscopic approach. Three consecutive patients undergoing transcanal endoscopic resection of middle ear paragangliomas using an UA were retrospectively studied. The primary outcome measure was achieving gross total tumor resection. Secondary outcomes included postoperative hearing and early convalescence. Two glomus tympanicum tumors and one small glomus jugulare were included, and complete tumor resection was achieved endoscopically in all cases...
February 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/28179334/potential-pitfalls-of-sdh-immunohistochemical-detection-in-paragangliomas-and-phaeochromocytomas-harbouring-germline-sdhx-gene-mutation
#15
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
#16
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...
February 6, 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28162975/comprehensive-molecular-characterization-of-pheochromocytoma-and-paraganglioma
#17
Lauren Fishbein, Ignaty Leshchiner, Vonn Walter, Ludmila Danilova, A Gordon Robertson, Amy R Johnson, Tara M Lichtenberg, Bradley A Murray, Hans K Ghayee, Tobias Else, Shiyun Ling, Stuart R Jefferys, Aguirre A de Cubas, Brandon Wenz, Esther Korpershoek, Antonio L Amelio, Liza Makowski, W Kimryn Rathmell, Anne-Paule Gimenez-Roqueplo, Thomas J Giordano, Sylvia L Asa, Arthur S Tischler, Karel Pacak, Katherine L Nathanson, Matthew D Wilkerson
We report a comprehensive molecular characterization of pheochromocytomas and paragangliomas (PCCs/PGLs), a rare tumor type. Multi-platform integration revealed that PCCs/PGLs are driven by diverse alterations affecting multiple genes and pathways. Pathogenic germline mutations occurred in eight PCC/PGL susceptibility genes. We identified CSDE1 as a somatically mutated driver gene, complementing four known drivers (HRAS, RET, EPAS1, and NF1). We also discovered fusion genes in PCCs/PGLs, involving MAML3, BRAF, NGFR, and NF1...
February 2, 2017: Cancer Cell
https://www.readbyqxmd.com/read/28158325/succinate-dehydrogenase-gene-mutation-with-cardiac-paraganglioma-multimodality-imaging-and-pathological-correlation
#18
Simone Romano, Cristiano Fava, Pietro Minuz, Afshin Farzaneh-Far
No abstract text is available yet for this article.
January 30, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28155290/the-diagnosis-of-bilateral-primary-renal-paragangliomas-in-a-cat
#19
Ryan B Friedlein, Alain J Carter, Robert D Last, Sarah Clift
A 9-year-old sterilised female domestic short-hair cat was referred with a history of vomiting and anorexia of 3 months' duration. Biochemistry, full-blood counts, thoracic radiographs, feline pancreatic-specific lipase, abdominal ultrasonography and feline immunodeficiency virus/feline leukaemia virus (FIV/FeLV) SNAP tests had been performed. Mild hypochloraemia and moderate hypokalaemia were evident on initial presentation. Abdominal ultrasonography initially revealed unilateral renal nodules on the left side...
January 24, 2017: Journal of the South African Veterinary Association
https://www.readbyqxmd.com/read/28149584/unusual-cause-of-massive-hemothorax-spontaneous-rupture-of-nonfunctioning-mediastinal-paraganglioma
#20
Feng Lin, Chengwu Liu, Lin Ma, Qiang Pu, Yunke Zhu, Zhilan Xiao, Chenglin Guo, Xiaolong Zhang, Chuan Li, Lunxu Liu
Mediastinal paraganglioma is a rare neurogenic tumor with a hypervascular feature. The spontaneous rupture of mediastinal paraganglioma is an unusual cause of massive hemothorax. Here we present a case of 39-year-old man with massive hemothorax due to the spontaneous rupture of a mediastinal paraganglioma. The man underwent successful resection of tumor and had an uneventful recovery. To our knowledge, this is the first reported case of spontaneous rupture of nonfunctioning mediastinal paraganglioma.
December 2016: Journal of Thoracic Disease
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