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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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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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Adrian F Daly, Emilie Castermans, Lindsey Oudijk, Mirtha A Guitelman, Pablo Beckers, Iulia Potorac, Sebastian J C M M Neggers, Nathalie Sacre, Aart-Jan van der Lely, Vincent Bours, Wouter W de Herder, and Albert Beckers

papillary thyroid cancer. She underwent total thyroidectomy, with clear lymph node and neck dissection. Given the history of tumors in three endocrine glands she underwent genetic testing for pheochromocytoma-paraganglioma, pituitary and MEN syndrome genes

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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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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

dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas . Journal of Clinical Endocrinology and Metabolism 92 3822 – 3828 . ( ) Bechmann N Poser I Seifert V

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E Rapizzi, T Ercolino, L Canu, V Giaché, M Francalanci, C Pratesi, A Valeri, and M Mannelli

–Lindau disease tumor suppressor gene . Science 260 1317 – 1320 . doi:10.1126/science.8493574 . Linnoila RI Keiser HR Steinberg SM Lack EE 1990 Histopathology of benign versus malignant sympathoadrenal paragangliomas: clinicopathologic study of

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Zoran Erlic, Ursula Ploeckinger, Alberto Cascon, Michael M Hoffmann, Laura von Duecker, Aurelia Winter, Gerit Kammel, Janina Bacher, Maren Sullivan, Berend Isermann, Lars Fischer, Andreas Raffel, Wolfram Trudo Knoefel, Matthias Schott, Tobias Baumann, Oliver Schaefer, Tobias Keck, Richard P Baum, Ioana Milos, Mihaela Muresan, Mariola Peczkowska, Andrzej Januszewicz, Kenko Cupisti, Anke Tönjes, Mathias Fasshauer, Jan Langrehr, Peter von Wussow, Abbas Agaimy, Günter Schlimok, Regina Lamberts, Thorsten Wiech, Kurt Werner Schmid, Alexander Weber, Mercedes Nunez, Mercedes Robledo, Charis Eng, Hartmut P H Neumann, and for the VHL-ICT Consortium and the German NET Registry

mortality. Major manifestations of VHL are hemangioblastoma of the retina and the central nervous system (CNS), renal clear cell carcinoma, pheochromocytoma and paraganglioma, multiple pancreatic cysts, ICTs, endolymphatic sac tumor of the inner ear, and

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D Cecchin, F Lumachi, M C Marzola, G Opocher, C Scaroni, P Zucchetta, F Mantero, and F Bui

(median 4.3 cm). None of the paragangliomas have proved to be malignant to date. The remaining 19 (28.4%) patients, not referred to surgery, underwent long-term (range 1–14 years, median 9.25 years) follow-up. Imaging (CT or MRI) performed in this

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Michael Solarski, Fabio Rotondo, William D Foulkes, John R Priest, Luis V Syro, Henriett Butz, Michael D Cusimano, and Kalman Kovacs

function . Nature Genetics 41 365 – 370 . ( ) 10.1038/ng.317 19219043 Mertens WC Grignon DJ Romano W 1993 Malignant paraganglioma with skeletal metastases and spinal cord compression: response and palliation with

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Jay S Fonte, Jeremyjones F Robles, Clara C Chen, James Reynolds, Millie Whatley, Alexander Ling, Leilani B Mercado-Asis, Karen T Adams, Victoria Martucci, Tito Fojo, and Karel Pacak

gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas . Journal of Clinical Endocrinology and Metabolism 92 3822 – 3828 . doi:10.1210/jc.2007-0709 . Bhatia KS Ismail MM Sahdev A Rockall AG Hogarth