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Samuel M O'Toole, Judit Dénes, Mercedes Robledo, Constantine A Stratakis, and Márta Korbonits

Introduction Pituitary adenomas (PA) and phaeochromocytomas/paragangliomas (phaeo/PGL) are relatively rare tumours. The prevalence of symptomatic PA in the general population is around 1 in 1000 ( Daly et al . 2006 , Fernandez et al . 2010 ). The

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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

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Margo Dona, Selma Waaijers, Susan Richter, Graeme Eisenhofer, Jeroen Korving, Sarah M Kamel, Jeroen Bakkers, Elena Rapizzi, Richard J Rodenburg, Jan Zethof, Marnix Gorissen, Gert Flik, Peter M T Deen, and Henri J L M Timmers

of a heterozygous SDH mutation are at risk of developing pheochromocytomas and paragangliomas (PPGLs), although usually a second hit in the form of a somatic mutation in the unaffected SDHB allele is required for the development of PPGLs. PPGLs

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Douglas Wiseman, James D McDonald, Dhaval Patel, Electron Kebebew, Karel Pacak, and Naris Nilubol

Introduction Pheochromocytomas and paragangliomas (PPGLs) are rare, catecholamine-secreting tumors arising from the chromaffin cells of the adrenal medulla and extra-adrenal neural crest tissues, which run from the skull base to the pelvis

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Arthur Varoquaux, Yann le Fur, Alessio Imperiale, Antony Reyre, Marion Montava, Nicolas Fakhry, Izzie-Jacques Namer, Guy Moulin, Karel Pacak, Maxime Guye, and David Taïeb

Introduction Paragangliomas (PGLs) are slow-growing hypervascular tumors arising from neural crest cell derivatives throughout the body. PGLs are closely aligned with the distribution of the autonomic nervous system and preferentially arise in the

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Tobias Else

Introduction The last 15 years have brought significant advances in our understanding of the genetic basis of hereditary syndromes with a predisposition to pheochromocytoma (PC) and paraganglioma (PGL) (PCPGL) development. In the 1990s the genetic

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

Introduction The Cancer Genome Atlas (TCGA) proposed that neuroendocrine tumors of adrenal paraganglia, pheochromocytomas (PCCs) and extra-adrenal paraganglia paragangliomas (PGLs, together denoted PPGL) can be divided into three main

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Aguirre A de Cubas, L Javier Leandro-García, Francesca Schiavi, Veronika Mancikova, Iñaki Comino-Méndez, Lucía Inglada-Pérez, Manuel Perez-Martinez, Nuria Ibarz, Pilar Ximénez-Embún, Elena López-Jiménez, Agnieszka Maliszewska, Rocío Letón, Álvaro Gómez Graña, Carmen Bernal, Cristina Álvarez-Escolá, Cristina Rodríguez-Antona, Giuseppe Opocher, Javier Muñoz, Diego Megias, Alberto Cascón, and Mercedes Robledo

Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors of neural crest origin. Up to an estimated 60% of PCCs/PGLs are associated with germline or somatic mutations in one many susceptibility genes ( RET

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Noriko Kimura, Ryoichi Takayanagi, Nae Takizawa, Eiji Itagaki, Takayuki Katabami, Narihiko Kakoi, Hiromi Rakugi, Yukihiro Ikeda, Akiyo Tanabe, Takeshi Nigawara, Sadayoshi Ito, Itaru Kimura, Mitsuhide Naruse, and The Phaeochromocytoma Study Group in Japan

Introduction Phaeochromocytomas (PHEO) of the adrenal gland and sympathetic paragangliomas (PGL) are catecholamine-producing tumours. Although 10–30% of these tumours metastasise, histopathological criteria that discriminate malignant from benign

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Roland Därr, Joan Nambuba, Jaydira Del Rivero, Ingo Janssen, Maria Merino, Milena Todorovic, Bela Balint, Ivana Jochmanova, Josef T Prchal, Ronald M Lechan, Arthur S Tischler, Vera Popovic, Dragana Miljic, Karen T Adams, F Ryan Prall, Alexander Ling, Meredith R Golomb, Michael Ferguson, Naris Nilubol, Clara C Chen, Emily Chew, David Taïeb, Constantine A Stratakis, Tito Fojo, Chunzhang Yang, Electron Kebebew, Zhengping Zhuang, and Karel Pacak

Introduction Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare catecholamine-producing neuroendocrine tumors (NETs) arising in or outside the adrenal medulla, respectively ( Lenders et al . 2005 ). By definition, a PHEO is an intra