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A Al-Sharefi, P Perros and R A James

Introduction Phaeochromocytomas/paragangliomas (PHAEO/PG) are chromaffin cell tumours, which can occur sporadically or as part of other hereditary syndromes including multiple endocrine neoplasia 2 (MEN-2), Von Hippel–Lindau syndrome (VHL) and

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Joakim Crona, Alberto Delgado Verdugo, Dan Granberg, Staffan Welin, Peter Stålberg, Per Hellman and Peyman Björklund

Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumours arising from chromaffin cells in adrenal medulla and autonomous ganglia. A majority of these tumours have a low proliferation and seldom metastasize. The understanding

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Natalie Rogowski-Lehmann, Aikaterini Geroula, Aleksander Prejbisz, Henri J L M Timmers, Felix Megerle, Mercedes Robledo, Martin Fassnacht, Stephanie M J Fliedner, Martin Reincke, Anthony Stell, Andrzej Januszewicz, Jacques W M Lenders, Graeme Eisenhofer and Felix Beuschlein

Introduction Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors that derive from adrenomedullary tissue (pheochromocytomas) in about 85% of cases and from extra-adrenal chromaffin tissue (paragangliomas) in the remainder

Open access

Xinlei Chen, Liru Hu, Caojie Liu, Guangcheng Ni and Yuwei Zhang

Introduction Pheochromocytoma and paraganglioma (PPGL) are tumors composed of chromaffin cells, which commonly produce one or more catecholamines: epinephrine, norepinephrine and dopamine. These tumors are rarely silent, and they are

Open access

Yiqiang Huang, Lin-ang Wang, Qiubo Xie, Jian Pang, Luofu Wang, Yuting Yi, Jun Zhang, Yao Zhang, Rongrong Chen, Weihua Lan, Dianzheng Zhang and Jun Jiang

Introduction Pheochromocytomas/paragangliomas (PCC/PGLs) are tumors, arose from neural crest-derived chromaffin cells, produce and secrete catecholamines ( 1 , 2 , 3 ). PCCs are tumors of the adrenal medulla and PGLs originate from

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Kranti Khadilkar, Vijaya Sarathi, Rajeev Kasaliwal, Reshma Pandit, Manjunath Goroshi, Gaurav Malhotra, Abhay Dalvi, Ganesh Bakshi, Anil Bhansali, Rajesh Rajput, Vyankatesh Shivane, Anurag Lila, Tushar Bandgar and Nalini S Shah

Introduction Malignant pheochromocytoma and paraganglioma (PCC/PGL) comprise approximately 10–20% of all pheochromocytomas and paragangliomas ( 1 ). According to the World Health Organization classification, the only accepted criterion for

Open access

Nicola Tufton, Lucy Shapiro, Anju Sahdev, Ajith V Kumar, Lee Martin, William M Drake, Scott A Akker and Helen L Storr

Introduction Almost 40–50% of all phaeochromocytomas (PCC) and paragangliomas (PGL) (together known as PPGLs) are now thought to be associated with germline mutations ( 1 , 2 ), but within the paediatric population this figure is higher (70

Open access

Caojie Liu, Qingguo Lv, Xinlei Chen, Guangcheng Ni, Liru Hu, Nanwei Tong and Yuwei Zhang

Introduction Pheochromocytomas and paragangliomas (PPGL) are rare catecholamine-secreting tumors with a low incidence of 0.8 per 100,000 individuals/year in the general population ( 1 ). However, the incidence among the hypertensive population

Open access

Katherine Van Loon, Li Zhang, Jennifer Keiser, Cendy Carrasco, Katherine Glass, Maria-Teresa Ramirez, Sarah Bobiak, Eric K Nakakura, Alan P Venook, Manisha H Shah and Emily K Bergsland

having a carcinoid tumor, pancreatic NET, high-grade neuroendocrine carcinomas (NECs), sympathetic paraganglioma or pheochromocytoma, or other NETs (adenocarcinoid tumors or adrenocortical carcinomas). The primary site of the tumor, functionality of the

Open access

Cristina Lamas, Elena Navarro, Anna Casterás, Paloma Portillo, Victoria Alcázar, María Calatayud, Cristina Álvarez-Escolá, Julia Sastre, Evangelina Boix, Lluis Forga, Almudena Vicente, Josep Oriola, Jordi Mesa and Nuria Valdés

the REGMEN (the Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytmas and Paragangliomas (PPGL)). Patients and methods The REGMEN is a collaborative and multicentric project designed by the Spanish Group for the Study of MEN and