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Jenny Welander, Peter Söderkvist, and Oliver Gimm

Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are neuroendocrine tumors that arise in the adrenal medulla or the extra-adrenal sympathetic and parasympathetic paraganglia ( DeLellis et al . 2004 ). Paraganglia are small organs

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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

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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

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Jean-Pierre Bayley, Marjan M Weiss, Anneliese Grimbergen, Bernadette T J van Brussel, Frederik J Hes, Jeroen C Jansen, Senno Verhoef, Peter Devilee, Eleonora P Corssmit, and Annette H J T Vriends

Introduction Paragangliomas of the head and neck occur as tumors of both the parasympathetically innervated head and neck paraganglia, most commonly in the carotid body, and of the sympathetic paraganglia and adrenal gland, frequently described as

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Angelica Malinoc, Maren Sullivan, Thorsten Wiech, Kurt Werner Schmid, Cordula Jilg, Joern Straeter, Serdar Deger, Michael M Hoffmann, Alexander Bosse, Gerd Rasp, Charis Eng, and Hartmut P H Neumann

recognized to confer a risk of RCC. von Hippel–Lindau disease (VHL) is associated with clear cell RCC (ccRCC) in about 30% of the affected patients. The other, pheochromocytoma/paraganglioma syndrome (PGL), associated with germline succinate dehydrogenase

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Valentina Piccini, Elena Rapizzi, Alessandra Bacca, Giuseppe Di Trapani, Raffaele Pulli, Valentino Giachè, Benedetta Zampetti, Emanuela Lucci-Cordisco, Letizia Canu, Elisa Corsini, Antongiulio Faggiano, Luca Deiana, Davide Carrara, Valeria Tantardini, Stefano Mariotti, Maria Rosaria Ambrosio, Maria Chiara Zatelli, Gabriele Parenti, Annamaria Colao, Carlo Pratesi, Giampaolo Bernini, Tonino Ercolino, and Massimo Mannelli

Introduction Paragangliomas of the head and neck region (HNPGLs) are hypervascular tumors arising from the neural crest cells. They are mostly found at the bifurcation of the common carotid artery where the carotid body is located, but they may also

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Maya B Lodish, Karen T Adams, Thanh T Huynh, Tamara Prodanov, Alex Ling, Clara Chen, Suzanne Shusterman, Camilo Jimenez, Maria Merino, Marybeth Hughes, Kendall W Cradic, Dragana Milosevic, Ravinder J Singh, Constantine A Stratakis, and Karel Pacak

medulla. While these structures normally degenerate and involute by adolescence, small clusters of chromaffin cells remain that can serve as sites of future tumor development. At least 135 cases of paragangliomas (PGLs) of the organ of Zuckerkandl have

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Hans K Ghayee, Bas Havekes, Eleonora P M Corssmit, Graeme Eisenhofer, Stephen R Hammes, Zahid Ahmad, Alexander Tessnow, Ivica Lazúrová, Karen T Adams, Antonio T Fojo, Karel Pacak, and Richard J Auchus

Introduction About 20% of catecholamine-producing tumors are derived from extra-adrenal chromaffin tissues and are termed paragangliomas (PGLs; Lenders et al . 2005 ). PGLs arise from chromaffin cells associated with sympathetic tissues. PGLs are

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Johanna Sandgren, Teresita Diaz de Ståhl, Robin Andersson, Uwe Menzel, Arkadiusz Piotrowski, Helena Nord, Martin Bäckdahl, Nimrod B Kiss, Michael Brauckhoff, Jan Komorowski, Henning Dralle, Ola Hessman, Catharina Larsson, Göran Åkerström, Carl Bruder, Jan P Dumanski, and Gunnar Westin

are therefore often termed as extra-adrenal pheochromocytomas or paragangliomas ( Erickson et al . 2001 , Bravo & Tagle 2003 , Karagiannis et al . 2007 ). Paraganglioma, however, refers to all neoplasias of the paraganglion nervous system

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Esther Korpershoek, Claudia K Stobbe, Francien H van Nederveen, Ronald R de Krijger, and Winand N M Dinjens

Introduction Pheochromocytomas (PCCs) are rare catecholamine-producing neuroendocrine tumors arising from chromaffin cells of the adrenal medulla. Extra-adrenal sympathetic paragangliomas (sPGLs) were previously known as extra-adrenal PCC. sPGLs are