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Valentina Piccini, Elena Rapizzi, Alessandra Bacca, Giuseppe Di Trapani, Raffaele Pulli, Valentino Giachè, Benedetta Zampetti, Emanuela Lucci-Cordisco, Letizia Canu, Elisa Corsini, Antongiulio Faggiano, Luca Deiana, Davide Carrara, Valeria Tantardini, Stefano Mariotti, Maria Rosaria Ambrosio, Maria Chiara Zatelli, Gabriele Parenti, Annamaria Colao, Carlo Pratesi, Giampaolo Bernini, Tonino Ercolino, and Massimo Mannelli

Introduction Paragangliomas of the head and neck region (HNPGLs) are hypervascular tumors arising from the neural crest cells. They are mostly found at the bifurcation of the common carotid artery where the carotid body is located, but they may also

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Goswin Y Meyer-Rochow, Nicole E Jackson, John V Conaglen, Denis E Whittle, Muthusamy Kunnimalaiyaan, Herbert Chen, Gunnar Westin, Johanna Sandgren, Peter Stålberg, Elham Khanafshar, Daniel Shibru, Quan-Yang Duh, Orlo H Clark, Electron Kebebew, Anthony J Gill, Rory Clifton-Bligh, Bruce G Robinson, Diana E Benn, and Stan B Sidhu

are caused by germ line mutations in genes associated with multiple endocrine neoplasia type 2, von Hippel–Lindau disease, and the pheochromocytoma/paraganglioma familial syndromes, but the remaining 76% are sporadic and the pathogenesis remains

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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

patients with pheochromocytoma and paraganglioma . Endocrine Connections 2 104 – 111 . ( doi:10.1530/EC-13-0009 ) Dionne JM Wu JK Heran M Murphy JJ Jevon G White CT 2006 Malignant hypertension, polycythemia, and

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Anna Angelousi, Georgios K Dimitriadis, Georgios Zografos, Svenja Nölting, Gregory Kaltsas, and Ashley Grossman

2009 Patient with malignant paraganglioma responding to the multikinase inhibitor sunitinib malate . Journal of Clinical Oncology 27 460 – 463 . ( doi:10.1200/JCO.2008.19.9380 ) Halperin DM Phan AT Hoff AO Aaron M Yao JC

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

dacarbazine treatment improves survival in patients with metastatic and unresectable malignant pheochromocytomas/paragangliomas . Hormones and Cancer 8 108 – 118 . ( ) 10.1007/s12672-017-0284-7 Assie G Letouze E

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Raquel G Martins, Joana B Nunes, Valdemar Máximo, Paula Soares, Joana Peixoto, Telmo Catarino, Teresa Rito, Pedro Soares, Luísa Pereira, Manuel Sobrinho-Simões, Ana Paula Santos, Joana Couto, Rui Henrique, Joana Matos-Loureiro, Paula Dias, Isabel Torres, and Jorge Lima

Dear Editor We would like to report a genetic screening of SDHB , SDHC , SDHD and SDHAF2 genes (hereafter abbreviated to SDHx ) in patients with paragangliomas (PGL) and phaeochromocytomas (PCC) from northern Portugal. PGL and PCC are

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Lucie Evenepoel, Raphaël Helaers, Laurent Vroonen, Selda Aydin, Marc Hamoir, Dominique Maiter, Miikka Vikkula, and Alexandre Persu

Dear Editor, Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from the adrenal medulla and extra-adrenal paraganglia, respectively. Forty percent are explained by germline mutations in known susceptibility

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

paragangliomas displaying increased malignant potential ( Neumann et al . 2004 , Timmers et al . 2007 ). Six cases of patients with an SDHB mutation who have both a PA and phaeo/PGL have been reported ( Table 1 ; Dénes et al . 2015 , Xekouki et al . 2015

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Esther Korpershoek, Nanne K Kloosterhof, Angelique Ziel-van der Made, Hanneke Korsten, Lindsey Oudijk, Jan Trapman, Winand N M Dinjens, and Ronald R de Krijger

patients with malignant pheochromocytomas or paragangliomas . Journal of Clinical Endocrinology and Metabolism 92 3822 – 3828 . ( doi:10.1210/jc.2007-0709 ). Bai F Pei XH Pandolfi PP Xiong Y 2006 p18 Ink4c and Pten constrain a positive

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Elizabeth Grubbs, Daniel Halperin, Steven G Waguespack, and Robert F Gagel

Overview The multiple endocrine neoplasia syndromes are a varied group of disorders that include multiple endocrine neoplasia types 1 and 2 (MEN 1, MEN 2), von Hippel-Lindau (VHL) disease , Carney complex (CC), hereditary pheochromocytoma/paraganglioma