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V H M Tsang, T Dwight, D E Benn, G Y Meyer-Rochow, A J Gill, M Sywak, S Sidhu, D Veivers, C M Sue, B G Robinson, R J Clifton-Bligh, and N R Parker

Introduction Pheochromocytomas (PCs) are catecholamine-secreting tumours of chromaffin cells of the adrenal medulla and are closely related to paragangliomas (PGLs) which arise from sympathetic and parasympathetic chains ( Favier et al . 2005

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Jean-Pierre Bayley and Peter Devilee

Introduction Paraganglioma–pheochromocytoma Paragangliomas and pheochromocytomas are neuroendocrine tumors that arise mainly in the adrenal medulla or paraganglia of the head and neck, but may also develop in abdominal or thoracic

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Sophie Moog, Charlotte Lussey-Lepoutre, and Judith Favier

Introduction Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from the adrenal medulla or extra-adrenal paraganglia, respectively. Paragangliomas can be found in the sympathetic nervous system of the thorax

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Rodrigo A Toledo, Yuejuan Qin, Subramanya Srikantan, Nicole Paes Morales, Qun Li, Yilun Deng, Sang-Woo Kim, Maria Adelaide A Pereira, Sergio P A Toledo, Xiaoping Su, Ricardo C T Aguiar, and Patricia L M Dahia

Introduction Pheochromocytomas and paragangliomas are sympathetic neural-derived neoplasms. Over one third of these tumors carry germline mutations in one of the ten distinct genes ( Jafri & Maher 2012 ). Remarkably, somatic events in

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Rodrigo Almeida Toledo

clinical features, i.e., renal carcinoma alone, renal carcinoma associated with pheochromocytoma, and pheochromocytoma alone ( Crossey et al. 1995 , Zbar et al. 1996 ). Pheochromocytomas and paragangliomas (PPGLs) are neural

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Ales Vicha, David Taieb, and Karel Pacak

these metabolic pathways or restoring altered pathways can lead to new approaches in cancer treatment. Pheochromocytoma and paraganglioma Pheochromocytomas (PHEOs)/paragangliomas (PGLs) are rare neuroendocrine tumors that produce catecholamines ( Lenders

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David Taïeb, Abhishek Jha, Giorgio Treglia, and Karel Pacak

Learning points Know the molecular taxonomy of pheochromocytoma and/or paraganglioma (PPGL) and the relationship between various genotypes and imaging phenotypes; Describe imaging features of SDHx -related PPGL; Understand

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Massimo Mannelli, Elena Rapizzi, Rossella Fucci, Letizia Canu, Tonino Ercolino, Michaela Luconi, and William F Young Jr

pheochromocytoma, termed ‘paraganglioma’, was also not linked to any germline mutations and paragangliomas located in the head and neck region (HN-paragangliomas) were considered as separate entities from those located in the abdomen, chest, and pelvis. In 2000

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Arthur S Tischler and Ronald R deKrijger

, Bausch et al . 2006 ), were known to be associated with pheochromocytomas, all with well-defined syndromic manifestations, and the genetic associations of extra-adrenal paragangliomas had not been determined. The main role of pathology was to make a

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Camilo Jimenez, Sasan Fazeli, and Alejandro Román-Gonzalez

Introduction Pheochromocytomas and paragangliomas are neuroendocrine tumors that make up less than 1% of all endocrine neoplasias. Pheochromocytomas and paragangliomas arise from the adrenal medulla and the extra-adrenal paraganglia