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M Montani, A M Schmitt, S Schmid, T Locher, P Saremaslani, P U Heitz, P Komminoth, and A Perren

. 2001 ) have recently been described in familial paragangliomas and pheochromocytomas. Several reasons make these genes candidate tumor suppressors for MTC: (1) allelic loss of the short and long arm of chromosome 1, the localization of SDHB and SDHC

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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

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Birke Bausch, Ulrich Wellner, Dirk Bausch, Francesca Schiavi, Marta Barontini, Gabriela Sanso, Martin K Walz, Mariola Peczkowska, Georges Weryha, Patrizia Dall'Igna, Giovanni Cecchetto, Gianni Bisogno, Lars C Moeller, Detlef Bockenhauer, Attila Patocs, Karoly Rácz, Dmitry Zabolotnyi, Svetlana Yaremchuk, Iveta Dzivite-Krisane, Frederic Castinetti, David Taieb, Angelica Malinoc, Ernst von Dobschuetz, Jochen Roessler, Kurt W Schmid, Giuseppe Opocher, Charis Eng, and Hartmut P H Neumann

Introduction Pheochromocytomas and paragangliomas are tumors of the overall paraganglial system, mainly the adrenal glands, the retroperitoneum, the pelvis, the thorax, and the skullbase and neck regions ( Neumann 2008 ). Such paraganglial tumors

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Lucia Martiniova, Shiromi M Perera, Frederieke M Brouwers, Salvatore Alesci, Mones Abu-Asab, Amanda F Marvelle, Dale O Kiesewetter, David Thomasson, John C Morris, Richard Kvetnansky, Arthur S Tischler, James C Reynolds, Antonio Tito Fojo, and Karel Pacak

Introduction Up to 36% of patients diagnosed with pheochromocytoma or paraganglioma will develop metastatic disease and have a 5-year survival of ∼50% ( O'Riordain et al . 1996 , Mundschenk & Lehnert 1998 , John et al . 1999 , Plouin et al

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Umasuthan Srirangalingam, Bernard Khoo, Lisa Walker, Fiona MacDonald, Robert H Skelly, Emad George, David Spooner, Linda B Johnston, John P Monson, Ashley B Grossman, W M Drake, Scott A Akker, Patrick J Pollard, Nick Plowman, Norbert Avril, Daniel M Berney, Jacky M Burrin, Rodney H Reznek, V K Ajith Kumar, Eamonn R Maher, and Shern L Chew

Introduction Recent studies have shown that ∼25% of apparently sporadic phaeochromocytomas and paragangliomas are due to germ line mutations in one of several familial syndrome genes, including von Hippel-Lindau ( VHL ), RET , succinate

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Jenny Welander, Adam Andreasson, Michael Brauckhoff, Martin Bäckdahl, Catharina Larsson, Oliver Gimm, and Peter Söderkvist

Introduction Pheochromocytomas and abdominal paragangliomas are catecholamine-producing tumors derived from neuroendocrine chromaffin cells in the adrenal medulla or the extra-adrenal paraganglia. The tumors can be a manifestation of different

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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

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Paraskevi Xekouki and Constantine A Stratakis

for the formation of multiple and possibly coexisting parasympathetic and sympathetic paragangliomas (PGLs) and/or pheochromocytomas (PHEOs) ( Baysal et al . 2000 , Astuti et al . 2001 ) made obsolete ( Dluhy 2002 ) at least one part of the axiom

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Eva Szarek, Evan R Ball, Alessio Imperiale, Maria Tsokos, Fabio R Faucz, Alessio Giubellino, François-Marie Moussallieh, Izzie-Jacques Namer, Mones S Abu-Asab, Karel Pacak, David Taïeb, J Aidan Carney, and Constantine A Stratakis

Introduction Carney triad (CTr) is a syndrome that describes the association of paragangliomas (PGLs) with gastrointestinal (GI) stromal tumors (GISTs) and pulmonary chondromas; other lesions, including pheochromocytomas, esophageal leiomyomas, and

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Asterios Karagiannis, Dimitri P Mikhailidis, Vasilios G Athyros, and Faidon Harsoulis

intraadrenal paraganglioma (PGL). Adrenal and extraadrenal PGLs produce significant amounts of catecholamines and give rise to the well-known clinical picture of PHEOs. In contrast, the parasympathetic PGLs (mainly in head and neck) rarely produce significant