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Anne-Paule Gimenez-Roqueplo, Mercedes Robledo, and Patricia L M Dahia

delays caused by the COVID-19 pandemic. In the intervening years, two international meetings related to pheochromocytomas (PCC) and paragangliomas (PGL) took place, Multiple Endocrine Neoplasia Workshop 2019 (in Houston) and 2021 (virtual). In 2019, the

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

Introduction Pituitary adenomas (PA) and phaeochromocytomas/paragangliomas (phaeo/PGL) are relatively rare tumours. The prevalence of symptomatic PA in the general population is around 1 in 1000 ( Daly et al . 2006 , Fernandez et al . 2010 ). The

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Margo Dona, Selma Waaijers, Susan Richter, Graeme Eisenhofer, Jeroen Korving, Sarah M Kamel, Jeroen Bakkers, Elena Rapizzi, Richard J Rodenburg, Jan Zethof, Marnix Gorissen, Gert Flik, Peter M T Deen, and Henri J L M Timmers

of a heterozygous SDH mutation are at risk of developing pheochromocytomas and paragangliomas (PPGLs), although usually a second hit in the form of a somatic mutation in the unaffected SDHB allele is required for the development of PPGLs. PPGLs

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Douglas Wiseman, James D McDonald, Dhaval Patel, Electron Kebebew, Karel Pacak, and Naris Nilubol

Introduction Pheochromocytomas and paragangliomas (PPGLs) are rare, catecholamine-secreting tumors arising from the chromaffin cells of the adrenal medulla and extra-adrenal neural crest tissues, which run from the skull base to the pelvis

Open access

Diana E Benn, Bruce G Robinson, and Roderick J Clifton-Bligh

Introduction Phaeochromocytomas (PCs) are tumours in the adrenal medulla, and paragangliomas (PGLs) arise in extra-adrenal sympathetic chromaffin tissue or head and neck parasympathetic tissues. Familial occurrence of PGLs was first reported in 1933

Open access

Camilo Jimenez, Bennett B Chin, Richard B Noto, Joseph S Dillon, Lilja Solnes, Nancy Stambler, Vincent A DiPippo, and Daniel A Pryma

Introduction Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells of the adrenal medulla and paraganglia, respectively ( Jimenez 2018 ). Each year, approximately 500–1600 new cases of PPGLs

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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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Joakim Crona, Angela Lamarca, Suman Ghosal, Staffan Welin, Britt Skogseid, and Karel Pacak

Introduction The Cancer Genome Atlas (TCGA) proposed that neuroendocrine tumors of adrenal paraganglia, pheochromocytomas (PCCs) and extra-adrenal paraganglia paragangliomas (PGLs, together denoted PPGL) can be divided into three main

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Tobias Else

Introduction The last 15 years have brought significant advances in our understanding of the genetic basis of hereditary syndromes with a predisposition to pheochromocytoma (PC) and paraganglioma (PGL) (PCPGL) development. In the 1990s the genetic

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Aguirre A de Cubas, L Javier Leandro-García, Francesca Schiavi, Veronika Mancikova, Iñaki Comino-Méndez, Lucía Inglada-Pérez, Manuel Perez-Martinez, Nuria Ibarz, Pilar Ximénez-Embún, Elena López-Jiménez, Agnieszka Maliszewska, Rocío Letón, Álvaro Gómez Graña, Carmen Bernal, Cristina Álvarez-Escolá, Cristina Rodríguez-Antona, Giuseppe Opocher, Javier Muñoz, Diego Megias, Alberto Cascón, and Mercedes Robledo

Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors of neural crest origin. Up to an estimated 60% of PCCs/PGLs are associated with germline or somatic mutations in one many susceptibility genes ( RET