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R Nawar and D Aron

reported higher frequency of AI in women may be an artifact related to their higher likelihood of undergoing imaging procedures. Patients with clinical features associated with functioning adrenal lesions (e.g., primary hyperaldosteronism, pheochromocytoma

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Rodrigo Almeida Toledo

familial syndrome, which is characterized by blood-formed tumors (hemangioblastomas), ccRCC and pancreatic neuroendocrine tumors, including pheochromocytomas ( Latif et al. 1993 ). Notably, specific germline VHL mutations are associated with distinct

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J Crona, F Beuschlein, K Pacak, and B Skogseid

12022-016-9452-5 ) 10.1007/s12022-016-9452-5 27709415 Rednam SP Erez A Druker H Janeway KA Kamihara J Kohlmann WK Nathanson KL States LJ Tomlinson GE Villani A , et al . 2017 Von Hippel-Lindau and Hereditary Pheochromocytoma/Paraganglioma Syndromes

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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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Jens Waldmann, Volker Fendrich, Julia Holler, Malte Buchholz, Ernst Heinmöller, Peter Langer, Annette Ramaswamy, Birgit Samans, Martin K Walz, Matthias Rothmund, Detlef K Bartsch, and Emily P Slater

K Brouwers FM Walther MM Linehan WM Munson PJ Mannelli M Goldstein DS Elkahloun AG 2004b Distinct gene expression profiles in norepinephrine- and epinephrine-producing hereditary and sporadic pheochromocytomas: activation of hypoxia

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Yulong Li and William F Simonds

bronchial NET (sometimes referred to as ‘carcinoid tumors’); and 3) MEN2A-associated medullary thyroid cancer and pheochromocytoma. Because of space limitations, we favor the citation of recent review articles, wherein the curious reader can easily find

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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

-0231 ) Eisenhofer G Huynh TT Pacak K Brouwers FM Walther MM Linehan WM Munson PJ Mannelli M Goldstein DS Elkahloun AG 2004 Distinct gene expression profiles in norepinephrine-and epinephrine-producing hereditary and sporadic pheochromocytomas

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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

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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

succinate, which acts as an oncometabolite ( Selak et al . 2005 ). We, and others, have recently shown that ex vivo metabolomics studies are very reliable methods for classifying various pheochromocytomas (PHEOs)/PGLs according to their genetic background

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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