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Paraganglioma

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https://www.readbyqxmd.com/read/28729379/functioning-metastatic-paraganglioma-of-the-urinary-bladder-in-a-10-year-old-child
#1
Alaeddine El Alayli, Mohammad Ali El Amine, Yaser El Hout, Lena Naffaa
Paragangliomas of the urinary bladder are very rare tumours representing less than 1% of bladder tumours. Preoperative diagnosis is essential to avoid perioperative complications related to catecholamine release. A high index of suspicion should be maintained when the classical symptoms of voiding-related paroxysms of headache, palpitation and dizziness are present. We present a rare case of malignant paraganglioma of the urinary bladder in a 10-year-old boy. The patient had the classic presentation. We review the radiological diagnostic tools and findings of this rare entity...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28728250/-paraganglioma-in-the-bottom-of-tongue-one-case-report
#2
H B Liu, P P Kang, S R Liu, J G Zhao, S H Zhang
No abstract text is available yet for this article.
July 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28720665/targeted-exome-sequencing-of-krebs-cycle-genes-reveals-candidate-cancer-predisposing-mutations-in-pheochromocytomas-and-paragangliomas
#3
Laura Remacha, Iñaki Comino-Méndez, Susan Richter, Laura Contreras, Maria Currás-Freixes, Guillermo Pita, Rocío Letón, Antonio Galarreta, Rafael Torres-Pérez, Emiliano Honrado, Scherezade Jiménez, Lorena Maestre, Sebastian Moran, Manel Esteller, Jorgina Satrústegui, Graeme Eisenhofer, Mercedes Robledo, Alberto Cascon
Purpose: Mutations in Krebs cycle genes are frequently found in patients with pheochromocytomas/paragangliomas. Disruption of SDH, FH or MDH2 enzymatic activities lead to accumulation of specific metabolites, which give rise to epigenetic changes in the genome that cause a characteristic hypermethylated phenotype. Tumors showing this phenotype, but no alterations in the known predisposing genes, could harbor mutations in other Krebs cycle genes. <p>Experimental Design: We used downregulation and methylation of RBP1, as a marker of a hypermethylation phenotype, to select eleven pheochromocytomas and paragangliomas for targeted exome sequencing of a panel of Krebs cycle-related genes...
July 18, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28719469/insm1-demonstrates-superior-performance-to-the-individual-and-combined-use-of-synaptophysin-chromogranin-and-cd56-for-diagnosing-neuroendocrine-tumors-of-the-thoracic-cavity
#4
Lisa M Rooper, Rajni Sharma, Qing Kay Li, Peter B Illei, William H Westra
Despite the importance of recognizing neuroendocrine differentiation when diagnosing tumors of the thoracic cavity, the sensitivity of traditional neuroendocrine markers is suboptimal, particularly for high-grade neuroendocrine carcinomas such as small cell lung carcinoma and large cell neuroendocrine carcinoma. To increase sensitivity, neuroendocrine markers are routinely ordered as panels of multiple immunostains where any single positive marker is regarded as sufficient evidence of neuroendocrine differentiation...
July 17, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28717527/vagal-paraganglioma-presenting-as-a-neck-mass-associated-with-cough-on-palpation
#5
Richard Heyes, Nizar Taki, Miriam A O'Leary
A 70-year-old female presented with a neck mass and sporadic dry cough, often leading to fits of coughing severe enough to cause vomiting. The patient reported that touching the mass triggered the cough. On examination, a 2.5 cm right-sided level two neck mass deep to the sternocleidomastoid was present. Palpation of the mass immediately triggered coughing. Cross-sectional imaging proposed vagal paraganglioma as the chief differential, which was confirmed following surgical excision. The patient reported complete resolution of her severe dry cough after surgery...
2017: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/28716765/sporadic-endolymphatic-sac-tumor-a-very-rare-cause-of-hearing-loss-tinnitus-and-dizziness
#6
Didde Trærup Schnack, Katalin Kiss, Søren Hansen, Hidemi Miyazaki, Birgitte Bech, Per Caye Thomasen
Sporadic endolymphatic sac tumor is a very rare neoplasm. It is low malignant, locally destructive and expansive, but non-metastasizing. The tumor is very rare in the sporadic form, but more often associated with Von Hippel-Lindau disease. A 65-year old man with left sided tinnitus and hearing loss for several months. Audiometry showed an asymmetrical sensory neural hearing loss on the left side up to 60 dB. The speech discrimination score was 46% and stapedial reflexes were absent. Several years earlier, he had suffered from periods of dizziness...
July 17, 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28715900/gangliocytic-paraganglioma-an-unusual-cause-of-upper-gastrointestinal-bleeding
#7
Yolanda Sánchez Torrijos, Rafael León Montañés, María Ángeles Mejías Manzano
Gangliocytic paraganglioma is an uncommon tumor, which is usually located in the ampulla of Vater, and may get confused in the differential diagnosis with ampuloma or GIST. Here we present a case of a patient with upper gastrointestinal bleeding as a predominant symptom, objectified by endoscopy an ulcerative polypoid mass at the juxtapilar level, with histological result of gangliocítica paraganglioma after multiple biopsies, and finally surgical resection. The interest of this case is the difficulty for diagnosing, the different treatments and prognosis that this implies, especially for the tumor location, as surgery can lead to great complications...
July 18, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28705916/biodistribution-and-dosimetry-of-18-f-meta-fluorobenzyl-guanidine-mfbg-a-first-in-human-pet-ct-imaging-study-of-patients-with-neuroendocrine-malignancies
#8
Neeta Pandit-Taskar, Pat B Zanzonico, Kevin D Staton, Jorge A Carrasquillo, Diane Reidy-Lagunes, Serge K Lyashchenko, Eva Burnazi, Hanwen Zhang, Jason S Lewis, Ronald Blasberg, Steven M Larson, Wolfgang Andreas Weber, Shakeel Modak
Introduction: Iodine-123-meta-iodobenzylguanidine ((123)I-MIBG) imaging is currently a mainstay in the evaluation of many neuroendocrine tumors, especially neuroblastoma. (123)I-MIBG imaging has several limitations that can be overcome by the use of a PET agent. (18)F-MFBG is a positron emission tomography (PET) analog of MIBG that may allow for single-day, high-resolution quantitative imaging. We conducted a first-in-human study of (18)F-MFBG PET imaging to evaluate the safety, feasibility, pharmacokinetics, and dosimetry of (18)F-MFBG in neuroendocrine tumors (NETs)...
July 13, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28704098/accuracy-of-plasma-free-metanephrines-in-the-diagnosis-of-pheochromocytoma-and-paraganglioma-a-systematic-review-and-meta-analysis
#9
Yan Chen, Huangmeng Xiao, Xieda Zhou, Xiaoyu Huang, Yanbing Li, Haipeng Xiao, Xiaopei Cao
OBJECTIVE: Various studies have validated plasma free metanephrines as biomarkers for pheochromocytoma and paraganglioma (PPGL). This meta-analysis aimed to estimate the overall diagnostic accuracy of this biochemical test for PPGL. METHODS: We searched the PubMed, the Cochrane Library, Web of Science, Embase, Scopus, OvidSP, and ProQuest Dissertations & Theses databases from January 1, 1995 to December 2, 2016 and selected studies written in English that assessed plasma free metanephrines in the diagnosis of PPGL...
July 13, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28704094/takotsubo-like-cardiomyopathy-in-a-large-cohort-of-patients-with-pheochromocytoma-and-paraganglioma
#10
Nadia Gagnon, Samer Mansour, Yoel Bitton, Isabelle Bourdeau
Pheochromocytoma (PHEO) and paraganglioma (PGL) (PPGL) may cause acute Takotsubo-like catecholamine cardiomyopathy (TLC). OBJECTIVE: To determine the prevalence and clinical presentation of TLC in a large cohort of patients with PPGL. METHODS: We reviewed retrospectively the records of consecutive patients with PPGL investigated in our center from 1995 to 2016. We collected clinical and paraclinical data of patients that had TLC in this cohort. We performed a literature review of cases of TTC-related to PPGL described previously from 1990-2015...
July 13, 2017: Endocrine Practice
https://www.readbyqxmd.com/read/28697655/functional-ulnar-nerve-paraganglioma-first-documented-occurrence-in-the-extremity-with-hitherto-undescribed-associated-extensive-glomus-cell-hyperplasia-and-tumorlet-formation
#11
Louis Tsun Cheung Chow, Michael Ho Ming Chan, Simon Kwok Chuen Wong
Extra-adrenal paraganglioma has never been described in the extremities. A 34-year-old woman complained of an enlarging mass in the right forearm for 18 months. Imaging showed a circumscribed vascular tumor attached to the ulnar nerve; biopsy revealed features of paraganglioma. The resected tumor consisted of zellballen pattern of chief cells staining positively for chromogranin with surrounding S100-positive sustentacular cells. The chief cells contained many neurosecretory granules and mitochondria, whereas the sustentacular cells contained a large amount of rough endoplasmic reticulum and some microfilaments...
July 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28694649/paraganglioma-of-the-filum-terminale-an-extremely-uncommon-neuroendocrine-neoplasm-located-in-spine
#12
Guru Dutta Satyarthee, Kanwaljit Garg, Sachin A Borkar
No abstract text is available yet for this article.
July 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28693800/review-of-sequencing-platforms-and-their-applications-in-phaeochromocytoma-and-paragangliomas
#13
REVIEW
Suja Pillai, Vinod Gopalan, Alfred King-Yin Lam
Genetic testing is recommended for patients with phaeochromocytoma (PCC) and paraganglioma (PGL) because of their genetic heterogeneity and heritability. Due to the large number of susceptibility genes associated with PCC/PGL, next-generation sequencing (NGS) technology is ideally suited for carrying out genetic screening of these individuals. New generations of DNA sequencing technologies facilitate the development of comprehensive genetic testing in PCC/PGL at a lower cost. Whole-exome sequencing and targeted NGS are the preferred methods for screening of PCC/PGL, both having precise mutation detection methods and low costs...
August 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28691673/gastric-paraganglioma-case-report-and-review-of-the-literature
#14
REVIEW
R Bura, Antonio Manca, R Ambu, G Pisano, Alessandro Melis, A Maturo, E Erdas, A Nicolosi, P G Calò
Very few cases of gastric paragangliomas have been reported in the literature to date. We report a rare case of parietal gastric paraganglioma fortuitously detected during intraoperative exploration. A 82-years-old woman presented to our emergency room for abdominal pain. On physical examination abdomen was painful on palpation and Blumberg's sign was present. The laboratory exams showed a neutrophilia in absence of leukocytosis. Acute appendicitis was suspected and a laparoscopy was performed. At exploration, the vermiform appendix was normal while a lumpy, hard-fibrous and white-pinkish extraluminal lesion of the anterior wall of the gastric body near the greater curvature of about 2 cm in diameter was present...
March 2017: Il Giornale di Chirurgia
https://www.readbyqxmd.com/read/28691354/feasibility-of-a-wait-and-scan-period-as-initial-management-strategy-for-head-and-neck-paraganglioma
#15
Thijs T G Jansen, Henri J L M Timmers, Henri A M Marres, Henricus P M Kunst
BACKGROUND: The main goal of head and neck paraganglioma (PGL) management is reduction of treatment-induced and tumor-induced complications. In the current study, tumor growth rates and tumor-induced complications during a wait-and-scan period are evaluated. METHODS: This was a retrospective cohort study. Tumor growth was measured in axial plane diameter and tumor volume. RESULTS: Of 59 jugulotympanic tumors, 71 carotid body tumors, and 29 vagal body tumors, 44% were growing (median follow-up of 63...
July 10, 2017: Head & Neck
https://www.readbyqxmd.com/read/28688875/diagnostic-surgical-and-postoperative-challenges-of-neuroendocrine-tumors-of-the-neck-clinical-experience-and-literature-review
#16
Mohammad Mussadaq Khan, Mohammad Iqbal Khan, Hussan Ali, Tehreem Kazmi, Hania Iqbal
BACKGROUND: Neuroendocrine tumors of the neck are rare swellings, which constitute about 0.03% of all tumors. Most are sporadically arising paragangliomas. Over 50% arise from the carotid bodies and pose difficulties in diagnosis and surgical management, which may result in disabling complications. OBJECTIVE: To determine the diagnostic, surgical and postoperative challenges, and how to overcome them, in the management of neuroendocrine tumors of the neck. STUDY DESIGN: Descriptive case series...
July 5, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28688872/paraganglioma-of-the-urinary-bladder-a-series-of-22-cases-in-a-single-center
#17
Hongyun Zhai, Xin Ma, Wenyuan Nie, Hongzhao Li, Cheng Peng, Xintao Li, Yu Zhang, Xu Zhang
OBJECTIVE: Paraganglioma of the urinary bladder (PUB) is rare in bladder cancer, and currently, a study examining large number of cases from a single center has not been reported. This study aimed to present the clinical features, treatments, pathological characteristics, and prognosis of PUB. PATIENTS AND METHODS: From January 2006 to June 2016, 22 patients were pathologically diagnosed with PUB in our hospital. Their clinical records, operative notes, pathological reports, and follow-up records were retrospectively reviewed...
March 28, 2017: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/28685646/giant-retroperitoneal-paraganglioma-debuting-with-gestational-hypertension
#18
Radu Dănilă, Dan-Nicolae Tesloianu, Daniel Timofte, Vlad Ghizdovat, Delia Ciobanu
No abstract text is available yet for this article.
July 7, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28685506/update-on-modern-management-of-pheochromocytoma-and-paraganglioma
#19
REVIEW
Jacques W M Lenders, Graeme Eisenhofer
Despite all technical progress in modern diagnostic methods and treatment modalities of pheochromocytoma/paraganglioma, early consideration of the presence of these tumors remains the pivotal link towards the best possible outcome for patients. A timely diagnosis and proper treatment can prevent the wide variety of potentially catastrophic cardiovascular complications. Modern biochemical testing should include tests that offer the best available diagnostic performance, measurements of metanephrines and 3-methoxytyramine in plasma or urine...
June 2017: Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28685225/phase-ii-trial-of-pazopanib-in-advanced-progressive-malignant-pheochromocytoma-and-paraganglioma
#20
Sina Jasim, Vera J Suman, Camilo Jimenez, Pamela Harris, Kostandinos Sideras, Jill K Burton, Francis Paul Worden, Richard J Auchus, Keith C Bible
INTRODUCTION: Pheochromocytomas and paragangliomas (Pheo/PGL) are rare, vascular, sometimes malignant endocrine tumors. Case reports indicate the activity of vascular endothelium growth factor receptor-targeted kinase inhibitors in these cancers. OBJECTIVES: To assess the antitumor activity and tolerability of pazopanib in progressive malignant Pheo/PGL. PATIENTS AND METHODS: This multicenter Phase II trial (MC107C) enrolled individuals  ≥18 years old with disease progression ≤ 6 months prior to registration, Eastern Cooperative Oncology Group PS 0-2, and measurable disease (response evaluation criteria in solid tumors 1...
August 2017: Endocrine
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