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L Fishbein, S Ben-Maimon, S Keefe, K Cengel, D A Pryma, A Loaiza-Bonilla, D L Fraker, K L Nathanson, and D L Cohen

, and dacarbazine treatment improves survival in patients with metastatic and unresectable malignant pheochromocytomas/paragangliomas . Hormones and Cancer 8 108 – 118 . ( doi:10.1007/s12672-017-0284-7 ) 10.1007/s12672-017-0284-7 Ayala

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Patricia L M Dahia, Roderick Clifton-Bligh, Anne-Paule Gimenez-Roqueplo, Mercedes Robledo, and Camilo Jimenez

future research projects for bridging these gaps. Diagnosis of ‘malignant’ pheochromocytoma and paraganglioma: state of the science Under the revised WHO classification (2018), pheochromocytomas and paragangliomas (PPGLs) are now referred to as

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Graeme Eisenhofer, Stefan R Bornstein, Frederieke M Brouwers, Nai-Kong V Cheung, Patricia L Dahia, Ronald R de Krijger, Thomas J Giordano, Lloyd A Greene, David S Goldstein, Hendrik Lehnert, William M Manger, John M Maris, Hartmut P H Neumann, Karel Pacak, Barry L Shulkin, David I Smith, Arthur S Tischler, and William F Young Jr

-adrenal paragangliomas remains a diagnostic challenge. Nevertheless, as reviewed by Arthur Tischler, several studies have attempted to distinguish benign from malignant pheochromocytoma using histopathological classifications that incorporate retrospective assessment of

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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

extended tumor panel of pheochromocytomas and paragangliomas. Notably and as our previous findings indicated a presence of CIMP in strong association with malignant paragangliomas with SDHB mutation ( Geli et al . 2008 , Kiss et al . 2008 ), the

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Noriko Kimura, Kazuhiro Takekoshi, Akira Horii, Ryo Morimoto, Tsuneo Imai, Yutaka Oki, Tomohito Saito, Sanae Midorikawa, Tadashi Arao, Chiho Sugisawa, Masanobu Yamada, Yuichi Otuka, Isao Kurihara, Kokichi Sugano, Minoru Nakane, Atsushi Fukuuchi, Takumi Kitamoto, Jun Saito, Tetsuo Nishikawa, and Mitsuhide Naruse

-Roqueplo AP 2007 Succinate dehydrogenase B 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 ). Benn DE

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Rodrigo Almeida Toledo

Broom R Freeman M Knox JJ 2009 Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma . Journal of Clinical Endocrinology and Metabolism 94 5 – 9 . ( doi:10.1210/jc.2008-1836 ) Kaelin WG

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Cristina Capatina, Georgia Ntali, Niki Karavitaki, and Ashley B Grossman

SL Evans A Broom R Freeman M Knox JJ 2009 Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma . Journal of Clinical Endocrinology and Metabolism 94 5 – 9 . ( doi:10.1210/jc.2008

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Thibault Bahougne, Alessio Imperiale, Gerlinde Averous, Gerard Chabrier, Nelly Burnichon, Anne Paule Gimenez-Roqueplo, Nassim Dali-Youcef, Rossella Libe, Eric Baudin, Catherine Roy, Herve Lang, and Laurence Kessler

Dear Editor, Pheochromocytoma (PHEO) and extra-adrenal paraganglioma (PGL) are neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the autonomic nervous system, respectively. These neoplasms are malignant in

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V Häyry, K Salmenkivi, J Arola, P Heikkilä, C Haglund, and H Sariola

malignant potential than phaeochromocytomas. Additionally, paragangliomas produce excessive amounts of noradrenaline, while phaeochromocytomas may produce just noradrenaline (i.e. von Hippel-Lindau tumours) or a combination of adrenaline and noradrenaline

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Maya B Lodish, Karen T Adams, Thanh T Huynh, Tamara Prodanov, Alex Ling, Clara Chen, Suzanne Shusterman, Camilo Jimenez, Maria Merino, Marybeth Hughes, Kendall W Cradic, Dragana Milosevic, Ravinder J Singh, Constantine A Stratakis, and Karel Pacak

patients with malignant pheochromocytomas or paragangliomas . Journal of Clinical Endocrinology and Metabolism 92 3822 – 3828 . Astuti D Latif F Dallol A Dahia PL Douglas F George E Skoldberg F Husebye ES Eng C Maher ER 2001