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