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Priya Gopie, Lin Mei, Anthony C Faber, Steven R Grossman, Steven C Smith, and Sosipatros A Boikos

, respectively. Collectively, these changes are thought to drive tumorigenesis in the multiple organ systems that demonstrate increased incidence of neoplasia in individuals harboring germline SDHx mutations, which include paragangliomas

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Atanaska Elenkova, Rabhat Shabani, Elena Kinova, Vladimir Vasilev, Assen Goudev, and Sabina Zacharieva

Introduction Cardiovascular disturbances are the major clinical manifestations of pheochromocytoma. Arterial hypertension is the leading symptom in approximately 90% of all patients, usually with a paroxysmal course, severe, malignant or

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Alessio Imperiale, Karim Elbayed, François-Marie Moussallieh, Nathalie Reix, Martial Piotto, Jean-Pierre Bellocq, Bernard Goichot, Philippe Bachellier, and Izzie-Jacques Namer

pathologies are adenomas (Ads) and adrenal cortical carcinomas (ACCs). While ACCs are relatively rare and extremely aggressive tumors, Ads are more frequently found and always benign. There is no evidence that Ads degenerate into malignant lesions ( Young 2007

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Ying Ni, Spencer Seballos, Shireen Ganapathi, Danielle Gurin, Benjamin Fletcher, Joanne Ngeow, Rebecca Nagy, Richard T Kloos, Matthew D Ringel, Thomas LaFramboise, and Charis Eng

neurodegenerative disease ( Bourgeron et al . 1995 , Parfait et al . 2000 ). In contrast, germline heterozygous mutations in the genes encoding the SDH subunits result in hereditary pheochromocytoma–paraganglioma syndrome ( Baysal et al . 2000 , Neumann et al

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Thomas G Papathomas, Lindsey Oudijk, Ellen C Zwarthoff, Edward Post, Floor A Duijkers, Max M van Noesel, Leo J Hofland, Patrick J Pollard, Eamonn R Maher, David F Restuccia, Richard A Feelders, Gaston J H Franssen, Henri J Timmers, Stefan Sleijfer, Wouter W de Herder, Ronald R de Krijger, Winand N M Dinjens, and Esther Korpershoek

pheochromocytomas (PCCs) and paragangliomas (PGLs) ( Vinagre et al . 2013 , Liu et al . 2014 ). In the current study, we examined the presence of these mutations in tumor types originating from the adrenal gland and extra-adrenal paraganglia including ACCs, PCCs

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Aristides Lytras and George Tolis

; MEN2B: #162 300; MEN4: #610 755), Carney complex (CNC; OMIM #160 980), von Recklinghausen disease (neurofibromatosis type 1, NF1; OMIM +162 200), VHLD, paraganglioma/pheochromocytoma syndromes (PGL1, 2, 3, 4; OMIM PGL1: #168 000; PGL2: %601 650; PGL3

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A Karpathakis, H Dibra, and C Thirlwell

hypermethylation ( Kiss et al . 2008 , 2013 ). CIMP positivity is present in 9% of paragangliomas and is associated with malignant behaviour ( Geli et al . 2008 ). Global hypomethylation and IGF2 promoter hypermethylation are associated with malignant

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Felix Haglund, Carl Christofer Juhlin, Taylor Brown, Mehran Ghaderi, Tiantian Liu, Adam Stenman, Andrii Dinets, Manju Prasad, Reju Korah, Dawei Xu, Tobias Carling, and Catharina Larsson

papillary, follicular, and anaplastic carcinomas of the thyroid, adrenocortical carcinomas, and paragangliomas. Interestingly, the mutations were found to be associated with telomerase activation, telomere length, malignant disease, patient age, and adverse

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F M Brouwers, E F Petricoin III, L Ksinantova, J Breza, V Rajapakse, S Ross, D Johann, M Mannelli, B L Shulkin, R Kvetnansky, G Eisenhofer, M M Walther, B A Hitt, T P Conrads, T D Veenstra, D P Mannion, M R Wall, G M Wolfe, V A Fusaro, L A Liotta, and K Pacak

Histopathology of benign versus malignant sympathoadrenal paragangliomas: clinicopathologic study of 120 cases including unusual histologic features. Human Pathology 21 1168 –1180. Liotta LA , Ferrari M & Petricoin E 2003

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Nicole Bechmann, Mats Leif Moskopp, Martin Ullrich, Bruna Calsina, Pål William Wallace, Susan Richter, Markus Friedemann, Katharina Langton, Stephanie M J Fliedner, Henri J L M Timmers, Svenja Nölting, Felix Beuschlein, Martin Fassnacht, Aleksander Prejbisz, Karel Pacak, Hans K Ghayee, Stefan R Bornstein, Peter Dieterich, Jens Pietzsch, Ben Wielockx, Mercedes Robledo, Nan Qin, and Graeme Eisenhofer

Introduction Pheochromocytomas and paragangliomas (PPGLs) are genetically heterogeneous tumors arising from the adrenal medulla or extra-adrenal paraganglia, respectively. A strong connection between genotype and phenotype has been recognized