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Open access

Diana E Benn, Bruce G Robinson, and Roderick J Clifton-Bligh

Introduction Phaeochromocytomas (PCs) are tumours in the adrenal medulla, and paragangliomas (PGLs) arise in extra-adrenal sympathetic chromaffin tissue or head and neck parasympathetic tissues. Familial occurrence of PGLs was first reported in 1933

Open access

Trisha Dwight, Edward Kim, Karine Bastard, Diana E Benn, Graeme Eisenhofer, Susan Richter, Massimo Mannelli, Elena Rapizzi, Aleksander Prejbisz, Mariola Pęczkowska, Karel Pacak, and Roderick Clifton-Bligh

frequently accompanied by illegitimate activation of hypoxia responses at normal oxygen levels (‘pseudohypoxia’, or the Warburg effect) ( Warburg 1956 , Ruan et al. 2009 ). Pheochromocytomas and paragangliomas (PPGLs) are tumors of the adrenal medulla

Open access

James F Powers, Brent Cochran, James D Baleja, Hadley D Sikes, Andrew D Pattison, Xue Zhang, Inna Lomakin, Annette Shepard-Barry, Karel Pacak, Sun Jin Moon, Troy F Langford, Kassi Taylor Stein, Richard W Tothill, Yingbin Ouyang, and Arthur S Tischler

adrenal medulla. Tumors called paragangliomas can arise anywhere in the distribution of normal paraganglia. By definition an intra-adrenal paraganglioma (PG) is called a pheochromocytoma (PC) ( Lloyd et al. 2017 ). At least 40% of pheochromocytomas and

Open access

J Crona, F Beuschlein, K Pacak, and B Skogseid

pheochromocytomas (PCCs) and paragangliomas (PGLs, collectively denoted PPGLs), 177 on adrenocortical tumors and 18 that fell into a general adrenal tumor category. In this review, we have referenced 110 of these manuscripts and selected three prominent topics that

Open access

Nimrod B Kiss, Andreas Muth, Adam Andreasson, C Christofer Juhlin, Janos Geli, Martin Bäckdahl, Anders Höög, Bo Wängberg, Ola Nilsson, Håkan Ahlman, and Catharina Larsson

Introduction Pheochromocytomas are catecholamine-secreting tumors of the chromaffin cells of the adrenal medulla. Extra-adrenal abdominal paragangliomas (here referred to as abdominal paraganglioma or paraganglioma) are related to neuroendocrine

Open access

Ruth T Casey, Benjamin G Challis, Alison Marker, Deborah Pitfield, Heok K Cheow, Ashley Shaw, Soo-Mi Park, Helen L Simpson, and Eamonn R Maher

Dear Editor, We have read with great interest the recent article by Tufton and coworkers reporting on the risk of metastasis in patients with paraganglioma (PGL) tumours associated with germline SDHA mutations ( Tufton et al . 2017 ). Herein

Open access

WenQi Yuan, WeiQinq Wang, Bin Cui, TingWei Su, Yan Ge, Lei Jiang, WeiWei Zhou, and Guang Ning

sympathetic nervous system. Both the clinical and biochemical features of pheochromocytomas are mostly the result of the overproduction of catecholamines. In sporadic pheochromocytomas and paragangliomas, ∼20% of them are associated with germline mutations

Open access

Martin Ullrich, Josephine Liers, Mirko Peitzsch, Anja Feldmann, Ralf Bergmann, Ulrich Sommer, Susan Richter, Stefan R Bornstein, Michael Bachmann, Graeme Eisenhofer, Christian G Ziegler, and Jens Pietzsch

Introduction Adrenal pheochromocytomas and extra adrenal paragangliomas (PPGLs) are rare catecholamine-producing tumors of chromaffin cell origin ( Lenders et al. 2005 , Harari & Inabnet 2011 , Jemal et al. 2011 ). In contrast to most

Open access

Tiantian Liu, Taylor C Brown, C Christofer Juhlin, Adam Andreasson, Na Wang, Martin Bäckdahl, James M Healy, Manju L Prasad, Reju Korah, Tobias Carling, Dawei Xu, and Catharina Larsson

pheochromocytoma (PCC) and an adrenocortical carcinoma (ACC). Pheochromocytomas (PCC) and abdominal paragangliomas (PGL) are typically catecholamine-producing tumors, originating from chromaffin cells in the paraganglia inside or outside the adrenal medulla ( Elder

Open access

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