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Clinical Consensus Guideline On The Management Of Phaeochromocytoma And Paraganglioma In Patients Harbouring Germline Sdhd Pathogenic Variants, David Taïeb, George B Wanna, Maleeha Ahmad, Charlotte Lussey-Lepoutre, Nancy D Perrier, Svenja Nölting, Laurence Amar, Henri J L M Timmers, Zachary G Schwam, Anthony L Estrera, Michael Lim, Erqi Liu Pollom, Lucas Vitzthum, Isabelle Bourdeau, Ruth T Casey, Frédéric Castinetti, Roderick Clifton-Bligh, Eleonora P M Corssmit, Ronald R De Krijger, Jaydira Del Rivero, Graeme Eisenhofer, Hans K Ghayee, Anne-Paule Gimenez-Roqueplo, Ashley Grossman, Alessio Imperiale, Jeroen C Jansen, Abhishek Jha, Michiel N Kerstens, Henricus P M Kunst, James K Liu, Eamonn R Maher, Daniele Marchioni, Leilani B Mercado-Asis, Ozgur Mete, Mitsuhide Naruse, Naris Nilubol, Neeta Pandit-Taskar, Frédéric Sebag, Akiyo Tanabe, Jiri Widimsky, Leah Meuter, Jacques W M Lenders, Karel Pacak

Journal Articles

Patients with germline SDHD pathogenic variants (encoding succinate dehydrogenase subunit D; ie, paraganglioma 1 syndrome) are predominantly affected by head and neck paragangliomas, which, in almost 20% of patients, might coexist with paragangliomas arising from other locations (eg, adrenal medulla, para-aortic, cardiac or thoracic, and pelvic). Given the higher risk of tumour multifocality and bilaterality for phaeochromocytomas and paragangliomas (PPGLs) because of SDHD pathogenic variants than for their sporadic and other genotypic counterparts, the management of patients with SDHD PPGLs is clinically complex in terms of imaging, treatment, and management options. Furthermore, locally aggressive disease can be discovered at a …