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https://www.readbyqxmd.com/read/28630023/epigenetics-of-pheochromocytoma-and-paraganglioma
#1
REVIEW
Peyman Björklund, Samuel Backman
Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors arising in the medullae of the adrenal glands or in paraganglia. The knowledge of the tumor biology of these lesions has increased dramatically during the past two decades and more than a dozen recurrently mutated genes have been identified. Different clusters have been described that share epigenetic signatures. Mutations in the succinate dehydrogenase complex subunit genes play a pivotal role in reprogramming the epigenetic state of these tumors by inhibiting epigenetic regulators such as TET enzymes and histone demethylases...
June 16, 2017: Molecular and Cellular Endocrinology
https://www.readbyqxmd.com/read/28626127/a-case-of-retroperitoneal-paraganglioma
#2
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/28620007/von-hippel-lindau-and-hereditary-pheochromocytoma-paraganglioma-syndromes-clinical-features-genetics-and-surveillance-recommendations-in-childhood
#3
REVIEW
Surya P Rednam, Ayelet Erez, Harriet Druker, Katherine A Janeway, Junne Kamihara, Wendy K Kohlmann, Katherine L Nathanson, Lisa J States, Gail E Tomlinson, Anita Villani, Stephan D Voss, Joshua D Schiffman, Jonathan D Wasserman
Von Hippel-Lindau disease (vHL) is a hereditary tumor predisposition syndrome that places affected individuals at risk for multiple tumors, which are predominantly benign and generally occur in the central nervous system or abdomen. Although the majority of tumors occur in adults, children and adolescents with the condition develop a significant proportion of vHL manifestations and are vulnerable to delayed tumor detection and their sequelae. Although multiple tumor screening paradigms are currently being utilized for patients with vHL, surveillance should be reassessed as the available relevant clinical information continues to expand...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28619735/the-evolving-role-of-succinate-in-tumor-metabolism-an-18-f-fdg-based-study
#4
Philippe Garrigue, Aurore Bodin-Hullin, Laure Balasse, Samantha Fernandez, Wassim Essamet, Françoise Dignat-George, Karel Pacak, Benjamin Guillet, David Taieb
Objective: In recent years, inherited and acquired mutations in the TCA cycle enzymes have been reported in diverse cancers. Pheochromocytomas and paragangliomas (PPGLs) often exhibit dysregulation of glucose metabolism which is also driven by mutations in genes encoding the TCA cycle enzymes or by activation of hypoxia signaling. PPGLs associated with succinate dehydrogenase (SDH) deficiency are characterized by high (18)F-fluorodeoxyglucose ([(18)F]-FDG) avidity. This association is currently only partially explained...
June 15, 2017: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/28616677/-preoperative-genetic-diagnostics-and-imaging-for-pediatric-pheochromocytoma-and-paraganglioma
#5
H Dralle
No abstract text is available yet for this article.
June 14, 2017: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/28611232/nuclear-imaging-in-a-cardiac-paraganglioma
#6
Nada H Almenieir, Shawn Karls, Vilma Derbekyan, Robert Lisbona
We report a case of a cardiac paraganglioma in the right atrioventricular groove, where the use of different nuclear medicine studies aided in the diagnosis and management.
June 13, 2017: Journal of Nuclear Medicine Technology
https://www.readbyqxmd.com/read/28607901/igg4-related-disease-igg4-rd-presenting-as-a-mass-in-the-carotid-triangle-masquerading-paraganglioma
#7
T Ramadass, V Balaji, S K J Sheba, K S Vali Ahmed, Raees Abdurahiman
IgG4-related disease (IgG4-RD) which is a protein disorder presented as a mass in the right carotid triangle in a 30 year male patient, who underwent battery of tests is described. The radiologist opined the mass as paraganglioma and the spindle shaped character of the mass also suggested neurogenic tumor in differential diagnosis. Reference to vascular surgeon also opined the same, and adviced for incisional biopsy. Histopathology report suggested IgG4-RD and immunochemistry confirmed the final diagnosis...
June 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28605453/malignant-pheochromocytoma-and-paraganglioma-272-patients-over-55-years
#8
Oksana Hamidi, William F Young, Nicole M Iñiguez-Ariza, Nana Esi Kittah, Lucinda Gruber, Cristian Bancos, Shrikant Tamhane, Irina Bancos
Context: Malignant pheochromocytoma (PHEO) and paraganglioma (PGL) are rare and knowledge of the natural history is limited. Objective: We aimed to describe baseline characteristics and outcomes of patients with malignant PHEO and PGL (PPGL) and to identify predictors of shorter survival. Design: Retrospective review of patients with malignant PPGL evaluated from 1960 to 2016. Setting: Referral center. Patients: The group comprised 272 patients...
June 12, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28605448/efficacy-of-peptide-receptor-radionuclide-therapy-prrt-for-functional-metastatic-paraganglioma-and-phaeochromocytoma
#9
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/28602102/metastatic-renal-cell-carcinoma-masquerading-as-jugular-foramen-paraganglioma-a-role-for-novel-magnetic-resonance-imaging
#10
Andrew J Thomas, Richard H Wiggins, Richard K Gurgel
OBJECTIVES: To describe a case of metastatic renal cell carcinoma (RCC) masquerading as a jugular foramen paraganglioma (JP). To compare imaging findings between skull base metastatic RCC and histologically proven paraganglioma. METHODS: A case of unexpected metastatic skull base RCC is reviewed. Computed tomography (CT) and magnetic resonance imaging (MRI) were compared between 3 confirmed cases of JP and our case of metastatic RCC. Diffusion-weighted MRI (DW-MRI) sequences and computed apparent diffusion coefficient (ADC) values were compared between these entities...
June 1, 2017: Annals of Otology, Rhinology, and Laryngology
https://www.readbyqxmd.com/read/28594934/effects-of-ppar%C3%AE-inhibition-in-head-and-neck-paraganglioma-cells
#11
Rosalba Florio, Laura De Lellis, Viviana di Giacomo, Maria Carmela Di Marcantonio, Loredana Cristiano, Mariangela Basile, Fabio Verginelli, Delfina Verzilli, Alessandra Ammazzalorso, Sampath Chandra Prasad, Amelia Cataldi, Mario Sanna, Annamaria Cimini, Renato Mariani-Costantini, Gabriella Mincione, Alessandro Cama
Head and neck paragangliomas (HNPGLs) are rare tumors that may cause important morbidity, because of their tendency to infiltrate the skull base. At present, surgery is the only therapeutic option, but radical removal may be difficult or impossible. Thus, effective targets and molecules for HNPGL treatment need to be identified. However, the lack of cellular models for this rare tumor hampers this task. PPARα receptor activation was reported in several tumors and this receptor appears to be a promising therapeutic target in different malignancies...
2017: PloS One
https://www.readbyqxmd.com/read/28591002/the-role-of-radiation-in-tympanojugular-paragangliomas-needs-to-be-objectively-re-evaluated
#12
Sampath Chandra Prasad, Mario Sanna
No abstract text is available yet for this article.
June 6, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28585398/genetic-status-determines-18-f-fdg-uptake-in-pheochromocytoma-paraganglioma
#13
Ankita Tiwari, Nalini Shah, Vijaya Sarathi, Gaurav Malhotra, Ganesh Bakshi, Gagan Prakash, Kranti Khadilkar, Reshma Pandit, Anurag Lila, Tushar Bandgar
INTRODUCTION: Although few studies have demonstrated utility of (18) F- fluoro-2-deoxy-d-glucose positron emission tomography/computerised tomography ((18) F-FDG PET/CT) in benign pheochromocytoma/paragangliomas (PCC/PGLs), there limited data on factors predicting the FDG uptake in PCC/PGL. METHODS: The study was conducted at a tertiary health care centre. In addition to the routine investigations, all patients (n = 96) with PCC/PGL were evaluated with (18) F-FDGPET/CT and majority (n = 78) underwent (131) I-metaiodobenzyl guanidine ((131) I-MIBG) scintigraphy...
June 5, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28584687/fluorine-18-fluorodihydroxyphenylalanine-positron-emission-tomography-scans-of-neuroendocrine-tumors-carcinoids-and-pheochromocytomas
#14
Italo Zanzi, Yana Studentsova, David Bjelke, Richard Warner, Barry Babchyck, Thomas Chaly
OBJECTIVES: Conventional methods of imaging neuroendocrine tumors with computed tomography, magnetic resonance imaging, indium-111-octreotide, or radiolabeled metaiodobenzilguanidine scintigraphy have limitations. This pilot study tried to improve the localization of these tumors with fluorine-18-fluorodihydroxyphenylalanine (F-DOPA) positron-emission tomography (PET) scanning. MATERIALS AND METHODS: We studied 22 patients, the majority of whom were referred with clinical diagnosis or suspicion of carcinoid (n = 11), neuroendocrine tumors (n = 7) or pheochromocytoma/paraganglioma (PGL) (n = 4)...
2017: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/28583225/the-two-hit-hypothesis-and-paraganglioma-of-the-head-and-neck
#15
Musheer Hussain, Edward Fisher, Robin Youngs, Jonathan Fishman
No abstract text is available yet for this article.
July 2017: Journal of Laryngology and Otology
https://www.readbyqxmd.com/read/28579473/duodenal-gangliocytic-paraganglioma-endoscopy-sonography-and-f-18-fdg-pet-ct-imaging
#16
Ming Yang, Jonathan A Leighton, Douglas O Faigel, Nitin N Katariya
No abstract text is available yet for this article.
May 18, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28570879/acute-onset-of-paraganglioma-of-filum-terminale-a-case-report-and-surgical-treatment
#17
Domenico Murrone, Bruno Romanelli, Giuseppe Vella, Aldo Ierardi
INTRODUCTION: Paragangliomas of filum terminale are rare benign tumors, arising from the adrenal medulla or extra-adrenal paraganglia. These lesions usually present with chronic back pain and radiculopathy and only two cases of acute neurological deficit have been reported in literature. PRESENTATION OF CASE: A case with an acute paraplegia and cauda equina syndrome due to an hemorrhagic paraganglioma of the filum terminale is described. Magnetic resonance imaging showed an intradural tumor extending from L1 to L2 compressing the cauda equina, with an intralesional and intradural bleed...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28570416/in-response-to-letter-to-the-editor-entitled-the-role-of-radiation-in-tympanojugular-paragangliomas-needs-to-be-re-evaluated
#18
Matthew L Bush, Mitchell R Dobberpuhl, Stevie Maxwell, Jonathan Feddock, William St Clair
No abstract text is available yet for this article.
May 31, 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/28566531/sdhb-mutation-carriers-with-malignant-pheochromocytoma-respond-better-to-cvd
#19
Lauren Fishbein, Sivan Ben-Maimon, Stephen Keefe, Keith Cengel, Daniel A Pryma, Arturo Loaiza-Bonilla, Douglas L Fraker, Katherine L Nathanson, Debbie L Cohen
Pheochromocytomas and paragangliomas (PCC/PGL) are tumors in the adrenal medulla and extra adrenal sites, respectively. About a quarter are metastatic, often with a long latency period. Some PCC/PGL are defined as clinically aggressive given extensive local invasion into adjacent normal tissue. These tumors, like metastatic PCC/PGL, cannot be surgically cured and patients can have continued secretion of excessive catecholamines with all related sequelae. For patients with metastatic or aggressive tumors, treatment options are limited...
May 31, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28566259/multimodal-approach-in-radioguided-surgery-in-a-case-of-multiple-paraganglioma
#20
N Sánchez, A Tapias, H Bowles, E Delgado, R Almenara, D Fuster, S Vidal-Sicart
The case involves a 34-year-old who underwent surgical removal of a retroperitoneal paraganglioma adjacent to the left kidney lower pole, and visualized by CT and MRI. The (123)I-MIBG scan was positive for this lesion and, in addition, another more caudal uptake, of smaller size and intensity, was observed at L5 level. It was not considered for removal, due to its lack of morphological definition. One week after the surgery, the patient presented with difficult-to-control high blood pressure. A second (123)I-MIBG scan was performed...
May 26, 2017: Revista Española de Medicina Nuclear e Imagen Molecular
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