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Paraganglioma

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https://www.readbyqxmd.com/read/28438335/an-unusual-case-of-mediastinal-paraganglioma-and-pituitary-adenoma
#1
Luis Gorospe, Alberto Cabañero-Sánchez, Gemma María Muñoz-Molina, Rubén Eduardo Pacios-Blanco, Almudena Ureña Vacas, Ernesto García-Santana
No abstract text is available yet for this article.
April 21, 2017: Surgery
https://www.readbyqxmd.com/read/28433725/clinical-characteristics-and-surgical-treatment-of-spinal-paraganglioma-a-case-series-of-18-patients
#2
Mengchen Yin, Quan Huan, Zhengwang Sun, Shaohui He, Ye Xia, Wen Mo, Junming Ma, Jianru Xiao
BACKGROUND AND OBJECTIVES: Paraganglioma rarely develops in the spine. With few cases reported, little knowledge about this disease was known. The objective of this study is to illustrate the clinical features, imaging manifestations, pathological appearances and long-term outcomes of the consecutive surgeries by literature review. METHODS: The clinical and follow-up data of 18 patients who were diagnosed of spinal paraganglioma and treated with surgeries in our hospitals from 2003 to 2014 were retrospectively analyzed...
March 30, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28432847/genotype-phenotype-correlation-in-paediatric-pheochromocytoma-and-paraganglioma-a-single-centre-experience-from-india
#3
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
#4
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/28431713/mediastinal-paraganglioma
#5
Stephanie N Buchanan, Kevin M Radecki, Lowell W Chambers
Paragangliomas of the mediastinum are rare, with only approximately 150 cases reported in the literature. Surgical excision is the treatment of choice; however, these tumors often lie near critical vascular structures. Here we present the case of a patient with a mediastinal paraganglioma discovered during a diagnostic procedure.
May 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28429830/establishment-and-evaluation-of-a-novel-biomarker-based-nomogram-for-malignant-phaeochromocytomas-and-paragangliomas
#6
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/28427002/incidental-posterior-mediastinal-paraganglioma-the-safe-approach-to-management-case-report
#7
Juan A Muñoz-Largacha, Roan J Glocker, Jacob Moalem, Michael J Singh, Virginia R Litle
INTRODUCTION: Paragangliomas are neuroendocrine tumors arising from chromaffin cells located in sympathetic paraganglia. Mediastinal paragangliomas are extremely rare and can be classified as functional or non-functional according to their ability for secreting catecholamines. Patients can be asymptomatic and the diagnosis is usually incidental. Complete surgical resection remains the standard of care for paragangliomas. PRESENTATION OF CASE: We present a 44-year-old woman with a functional mediastinal paraganglioma incidentally found during the perioperative imaging workup for a diagnosed breast carcinoma...
March 31, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28423608/anthracyclines-suppress-pheochromocytoma-cell-characteristics-including-metastasis-through-inhibition-of-the-hypoxia-signaling-pathway
#8
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/28414676/pheochromocytoma-of-the-organ-zuckerkandl
#9
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/28413667/paraganglioma-of-the-bladder-in-a-kidney-transplant-recipient-a-case-report
#10
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/28412985/active-surveillance-management-of-head-and-neck-paragangliomas-case-series-and-review-of-the-literature
#11
L Harrison, R Corbridge
BACKGROUND: Head and neck paragangliomas are rare. They are usually slow-growing, benign, non-catecholamine secreting tumours, traditionally treated with surgical excision. Complications of surgical excision include lower cranial nerve palsies, stroke and death. METHOD: A retrospective case note analysis was conducted of patients with head and neck paragangliomas treated with a watch-and-scan policy from March 2003 to September 2015, and the relevant literature was reviewed...
April 17, 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/28412254/percutaneous-coronary-intervention-for%C3%A2-treatment-of-paraganglioma-with%C3%A2-coronary-vascularization
#12
Rocío González-Ferreiro, Emilio Rodriguez-Ruiz, Diego López-Otero, Amparo Martínez Monzonís, María Álvarez Barredo, Ramiro Trillo-Nouche, José Ramón González Juanatey
No abstract text is available yet for this article.
April 6, 2017: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28412079/polymyalgia-rheumatica-and-vagal-paraganglioma
#13
V L'Huillier, O Mauvais, S Valmary-Degano, L Tavernier
INTRODUCTION: Vagal paraganglioma are rare tumors that are mostly asymptomatic. We report a case of vagal paraganglioma associated with paraneoplastic polymyalgia rheumatica and review the literature on benign paragangliomas of the head and neck associated with paraneoplastic syndrome. CASE REPORT: A 53-year-old man presented with atypical polymyalgia rheumatica. MRI revealed a tumor that was then surgically excised. Histological examination confirmed the diagnosis of benign vagal paraganglioma...
April 12, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/28409892/failure-of-mibg-scan-to-detect-metastases-in-sdhb-mutated-pediatric-metastatic-pheochromocytoma
#14
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/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
#15
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/28401029/iatrogenic-central-retinal-artery-occlusion-after-carotid-body-tumor-embolization-and-excision
#16
Carlos M Rangel, Sergio Jaramillo, Clara L Varón, Angélica M Prada
Objective: To report a case of iatrogenic central retinal artery occlusion after embolization and surgical resection of carotid body paraganglioma. Methods: Case report Results: One adult female patient presented with persistent unilateral visual loss after embolization with Embosphere(®) and Contour(®) microparticles of carotid body tumor. Fluorescein angiography revealed intraluminal microspheres in the central retinal artery ramifications. OCT revealed intraretinal spherical, hyporeflective particles with posterior shadowing...
2017: GMS Ophthalmology Cases
https://www.readbyqxmd.com/read/28392094/perioperative-management-of-pheochromocytoma
#17
REVIEW
Julian Naranjo, Sarah Dodd, Yvette N Martin
Pheochromocytomas are rare neuroendocrine tumors that produce and store catecholamines. Without adequate preparation, the release of excessive amounts of catecholamines, especially during anesthetic induction or during surgical removal, can produce life-threatening cardiovascular complications. This review focuses on the perioperative management of pheochromocytoma/paragangliomas, initially summarizing the clinical aspects of the disease and then highlighting the current evidence available for preoperative, intraoperative, and postoperative anesthetic management...
February 4, 2017: Journal of Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28387021/diagnosing-clear-cell-neuroendocrine-tumors-on-cytological-specimens-report-of-two-cases-and-brief-literature-review
#18
Kaila Buckley, Zaibo Li
Although many of the common manifestations of Von Hippel-Lindau (VHL) disease are relatively well known, there is one particular entity which is quite rare, but which appears to show a specific predilection for VHL patients. This entity is clear cell neuroendocrine tumor (NET). It is a difficult diagnosis to make due to its cytomorphologic similarities to other entities, such as metastatic clear cell renal cell carcinoma and paraganglioma, amongst others. These tumors, however, are characteristically positive for neuroendocrine markers such as synaptophysin and chromogranin, as well as cytokeratins...
April 6, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28386672/malignancy-in-pheochromocytoma-or-paraganglioma-integrative-analysis-of-176-cases-in-tcga
#19
Yong Joon Suh, Ji-Young Choe, Hyo Jin Park
Methods of diagnosing malignant pheochromocytoma (PCC) or paraganglioma (PGL) are needed. However, there are no reliable histopathologic criteria to distinguish malignant PCC/PGLs. The recent genomic analysis of The Cancer Genome Atlas (TCGA) provides in-depth information enabling more accurate diagnosis of disease entities. Therefore, we investigated genomic expression differences and mutational differences of malignant PCC/PGLs with TCGA. As of December 2014, TCGA had acquired multigenomic analysis of 176 PCC/PGL samples...
April 6, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28384794/clinical-characterization-of-the-pheochromocytoma-and-paraganglioma-susceptibility-genes-sdha-tmem127-max-and-sdhaf2-for-gene-informed-prevention
#20
Birke Bausch, Francesca Schiavi, Ying Ni, Jenny Welander, Attila Patocs, Joanne Ngeow, Ulrich Wellner, Angelica Malinoc, Elisa Taschin, Giovanni Barbon, Virginia Lanza, Peter Söderkvist, Adam Stenman, Catharina Larsson, Fredrika Svahn, Jin-Lian Chen, Jessica Marquard, Merav Fraenkel, Martin A Walter, Mariola Peczkowska, Aleksander Prejbisz, Barbara Jarzab, Kornelia Hasse-Lazar, Stephan Petersenn, Lars C Moeller, Almuth Meyer, Nicole Reisch, Arnold Trupka, Christoph Brase, Matthias Galiano, Simon F Preuss, Pingling Kwok, Nikoletta Lendvai, Gani Berisha, Özer Makay, Carsten C Boedeker, Georges Weryha, Karoly Racz, Andrzej Januszewicz, Martin K Walz, Oliver Gimm, Giuseppe Opocher, Charis Eng, Hartmut P H Neumann
Importance: Effective cancer prevention is based on accurate molecular diagnosis and results of genetic family screening, genotype-informed risk assessment, and tailored strategies for early diagnosis. The expanding etiology for hereditary pheochromocytomas and paragangliomas has recently included SDHA, TMEM127, MAX, and SDHAF2 as susceptibility genes. Clinical management guidelines for patients with germline mutations in these 4 newly included genes are lacking. Objective: To study the clinical spectra and age-related penetrance of individuals with mutations in the SDHA, TMEM127, MAX, and SDHAF2 genes...
April 6, 2017: JAMA Oncology
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