ReviewClinical consensus guideline on the management ofphaeochromocytoma and paraganglioma in patientsharbouring germline SDHD pathogenic variantsDavid Taieb,George B Wanna,Maleeha Ahmad Charlotte Lussey-Lepoutre,NancyD Perier,Svenja Nolting,LaurenceAmar,HenriJLM Timmers,Zachary G Schwam,AnthonyL Estrera,Michael Lim,Ergi Liu Pollom,Lucas Vitzthum,Isabelle Bourdeau,Ruth T Casey.Frederic Castinetti,Roderick Clifton-Bligh,Eleonora P M Corssmit,Ronald R de Krijger,Jlaydira Del Rivero,Graeme Eisenhofer,Hans K Ghayee,Anne-Paule Gimenez-Rogueplo,Ashley Grossman,Alessio Imperiale,Jeroen CJansen,Abhishek ha,Michiel N Kerstens,Henricus PM Kunst,James K Liu,Eamonn R Maher,Daniele Marchioni Leilani B Mercado-Asis,Ozgur Mete,Mitsuhide Naruse,Naris Nilubol Neeta Pandit-Taskar,Frederic Sebag.Akiyo Tanabe,Jiri Widimsky,Leah Meuter,JacquesW M Lenders,Karel PacakPatients with germline SDHD pathogenic variants (encoding succinate dehydrogenase subunit D;ie,LancetDiabetes Endocrinol2023paraganglioma 1 syndrome)are predominantly affected by head and neck paragangliomas,which,in almost 20%Published Onlineof patients,might coexist with paragangliomas arising from other locations (eg,adrenal medulla,para-aortic,Mch31.2023cardiac or thoracic,and pelvic).Given the higher risk of tumour multifocality and bilaterality forhttps/idaigrg/101016452213858723100028.4phaeochromocytomas and paragangliomas(PPGLs)because of SDHD pathogenic variants than for their sporadicDepartment of Neurosurgeryand other genotypic counterparts,the management of patients with SDHD PPGLs is clinically complex in terms(M Ahmad MD.Poof MLim MD).of imaging,treatment,and management options.Furthermore,locally aggressive disease can be discovered atDepartment of Physiciana young age or late in the disease course,which presents challenges in balancing surgical intervention with variousAssistant Studies (L Meuter BS)medical and radiotherapeutic approaches.The axiom-first,do no harm-should always be considered and anand Department ofRadiationinitial period of observation(ie,watchful waiting)is often appropriate to characterise tumour behaviour in patientsOncology (E L Pollom MD,LVitzthumMD),Sta nfordwith these pathogenic variants.These patients should be referred to specialised high-volume medical centres.ThisUniversity Schoolof Medicine,consensus guideline aims to help physicians with the clinical decision-making process when caring for patientsPalo Alta.CA.USA:Universitewith SDHD PPGLs.ParisCite,Inserm,PARCCEquipe Labellisee parla Liguecontre le Cancer,Paris,FranceIntroductionMethods(Prof LAmar MD,Hereditary head andneck paragangliomas(HNPGLs)are The consensus induded three chairpersons (DT,Prof A-P Gimenez-Roqueplo MD,the most common tumours in patients with gemmlineJWML and KP)and one project manager (LM).TheProf CLussey-Lepoutre MD):SDHD (encoding succinate dehydrogenase subunit D)Unite dhypertension arterielleproject was initiated in May,2021,and started with(Prof LAmar)and Departementpathogenic varian
