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Florian Haller, Evgeny A Moskalev, Fabio R Faucz, Sarah Barthelmeß, Stefan Wiemann, Matthias Bieg, Guillaume Assie, Jerome Bertherat, Inga-Marie Schaefer, Claudia Otto, Eleanor Rattenberry, Eamonn R Maher, Philipp Ströbel, Martin Werner, J Aidan Carney, Arndt Hartmann, Constantine A Stratakis, and Abbas Agaimy

Introduction Carney triad (CT) is a rare condition with synchronous or metachronous occurrence in a patient of three tumor entities, namely gastric gastrointestinal stromal tumor (GIST), paraganglioma (PGL), and pulmonary chondroma ( Carney et al

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E Kim, E M Rath, V H M Tsang, A P Duff, B G Robinson, W B Church, D E Benn, T Dwight, and R J Clifton-Bligh

Introduction Phaeochromocytoma (PC) and paraganglioma (PGL) are rare neuroendocrine tumours that arise from adrenal glands and extra-adrenal sites respectively ( Amar et al . 2005 , Gimenez-Roqueplo et al . 2012 ). These tumours occur in the

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Cristina Capatina, Georgia Ntali, Niki Karavitaki, and Ashley B Grossman

Introduction Paragangliomas (PGLs) are rare tumours originating from paraganglia – small groups of neuroendocrine cells arising from the autonomic nervous system ganglia. The sympathetic paraganglia are mostly located along the sympathetic nerve

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B Havekes, A A van der Klaauw, M M Weiss, J C Jansen, A G L van der Mey, A H J T Vriends, B A Bonsing, J A Romijn, and E P M Corssmit

Introduction Paragangliomas are frequently multifocal tumors arising from the neural crest cells associated with the autonomic nervous system. Traditionally, they are divided into head-and-neck paragangliomas (HNP) and paragangliomas located in the

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Ernst von Dobschuetz, Helena Leijon, Camilla Schalin-Jäntti, Francesca Schiavi, Michael Brauckhoff, Mariola Peczkowska, Giovanna Spiazzi, Serena Demattè, Maria Enrica Cecchini, Paola Sartorato, Jolanta Krajewska, Kornelia Hasse-Lazar, Katarzyna Roszkowska-Purska, Elisa Taschin, Angelica Malinoc, Lars A Akslen, Johanna Arola, Dariusz Lange, Ambrogio Fassina, Gianmaria Pennelli, Mattia Barbareschi, Jutta Luettges, Aleksander Prejbisz, Andrzej Januszewicz, Tim Strate, Birke Bausch, Frederic Castinetti, Barbara Jarzab, Giuseppe Opocher, Charis Eng, and Hartmut P H Neumann

Introduction Neuroendocrine tumors of the thyroid gland are rare, and virtually all are medullary thyroid carcinoma ( Ferri et al . 2009 ). Paragangliomas of the head and neck are neuroendocrine neoplasms developing from parasympathetic paraganglia

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Frédéric Castinetti, Alexander Kroiss, Rakesh Kumar, Karel Pacak, and David Taieb

Current approaches for localization of pheochromocytomas/paragangliomas Paragangliomas associated with the parasympathetic nervous system Glomus tumors and other paragangliomas (PGLs) of parasympathetic origin develop from non-chromaffin organs that

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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

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Bora E Baysal and Eamonn R Maher

Introduction Paragangliomas are neuroendocrine neoplasms that may arise from parasympathetic or sympathetic paraganglia. In general, those arising from parasympathetic paraganglia are non-secretory, associated with head and neck paraganglia and are

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Henri J L M Timmers, Anne-Paule Gimenez-Roqueplo, Massimo Mannelli, and Karel Pacak

Introduction Paragangliomas (PGLs) derive from either sympathetic tissue in adrenal and extra-adrenal locations or from parasympathetic tissue of the head and neck ( DeLellis et al . 2004 ). A PGL arising from chromaffin cells of the adrenal

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Alexandra Chrisoulidou, Gregory Kaltsas, Ioannis Ilias, and Ashley B Grossman

Introduction Phaeochromocytomas are tumours arising from chromaffin tissue of the adrenal medulla, whereas paragangliomas are chromaffin-cell tumours located at extra-adrenal sites along the sympathetic and/or the parasympathetic