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Bora E Baysal and Eamonn R Maher

nervous system paraganglia usually arise in the abdomen and thorax, and secrete catecholamines ( Tischler 2008 ). Thus they are functionally and histologically similar to pheochromocytomas (in older literature they were often described as extra

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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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Andreas Machens and Henning Dralle

Introduction More than fifty years ago, the medical community witnessed the birth of a new hereditary endocrine syndrome encompassing medullary thyroid cancer (MTC) and pheochromocytoma: ‘Sipple syndrome’, named after the author of this

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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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Ales Vicha, David Taieb, and Karel Pacak

Cubas AA Amar L Barontini M 2012b MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma . Clinical Cancer Research 18 2828 – 2837 . ( doi:10.1158/1078-0432.CCR-12-0160 ). Cairns RA Harris IS Mak TW 2011

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Roeliene C Kruizinga, Wim J Sluiter, Elisabeth G E de Vries, Bernard A Zonnenberg, Cornelis J Lips, Anouk N A van der Horst-Schrivers, Annemiek M E Walenkamp, and Thera P Links

discriminators of different hereditary forms of pheochromocytoma . Clinical Chemistry 57 411 – 420 . ( doi:10.1373/clinchem.2010.153320 ). Eisenhofer G Timmers HJ Lenders JW Bornstein SR Tiebel O Mannelli M King KS Vocke CD Linehan WM Bratslavsky G 2011b Age at

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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

mutation carriers among pheochromocytoma and paraganglioma patients is of clinical importance but in principle requires extensive mutation screenings in a large group of patients – as several genes are involved, and hereditary predisposition is often

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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

Introduction A pheochromocytoma is a rare endocrine tumour predominately arising from the catecholamine-producing chromaffin cells in the adrenal medulla. In 10–23% of cases, tumours occur in extra-adrenal locations in sympathetic paraganglia, and

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Camilo Jimenez, Sasan Fazeli, and Alejandro Román-Gonzalez

mitochondrial signals in pheochromocytomas . PLoS Genetics 1 72 – 80 . ( https://doi.org/10.1371/journal.pgen.0010008 ) Dahia PLM Clifton-Bligh R Gimenez-Roqueplo A-P Robledo M Jimenez C 2020 HEREDITARY ENDOCRINE TUMOURS: CURRENT STATE

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Joakim Crona, Angela Lamarca, Suman Ghosal, Staffan Welin, Britt Skogseid, and Karel Pacak

Timmers H Mannelli M Grebe SK Hofbauer LC Bornstein SR Tiebel O Adams K Bratslavsky G , 2011a Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma . Clinical