INTERNATIONAL JOURNAL OFCheck for updatesJROnternational Journal of Urology (2023)doi:10.1111/ju.15213GuidelinesClinical Practice Guidelines for tuberous sclerosis complex-associated renal angiomyolipoma by the Japanese UrologicalAssociation:Summary of the updateTakahiro Osawa,Mototsugu Oya,2 Tohru Okanishi,3 Ryohei Kuwatsuru,4 Haruna Kawano,sYoshihiko Tomita,Yo Nida,?Norio Nonomura,Takashi Hatano,Yasuhisa Fujii,10Masashi Mizuguchill and Nobuo Shinohara2Department of Urology,Hokkaido University Hospital,Sapporo,Japan,Department of Urology,Keio University School ofMedicine,Tokyo,Japan,Division of Child Neurology,Department of Brain and Neurosciences,Faculty of Medicine,TottoriUniversity,Yonago,Japan,Department of Radiology,School of Medicine and Graduate School of Medicine,Juntendo University,Tokyo,Japan,5Department of Urology,Juntendo University Graduate School of Medicine,Tokyo,Japan,Department of Urologyand Department of Molecular Oncology,Niigata University Graduate School of Medicine,Niigata,Japan,Center for ClinicalGenomics,Kanazawa Medical University Hospital,Uchinada,Japan,Department of Urology,Osaka University Graduate School ofMedicine,Suita,Japan,Department of Urology,Seirei Yokohama Hospital,Yokohama,Japan,Department of Urology,TokyoMedical and Dental University,Tokyo,Japan,Department of Pediatrics,National Rehabilitation Center for Children withDisabilities,Tokyo,Japan,and 2Department of Renal and Genitourinary Surgery,Hokkaido University Graduate School ofMedicine,Sapporo,JapanAbbreviations AcronymsNew clinical issues have been raised through an interval of 7 years from the previousAML angiomyolipomaversion (2016).In this study,we update the "Clinical Practice Guidelines for tuberousBQ Background Questionssclerosis complex-associated renal angiomyolipoma"as a 2023 version under guidanceCO Clinical Questionsby the Japanese Urological Association.The present guidelines were cooperativelyFQ Future Questionsprepared by the Japanese Urological Association and Japanese Society of TuberousLAM=Sclerosis Complex;committee members belonging to one of the two societies orlymphangioleiomyomatosisspecializing in the treatment of this disease were selected to prepare the guidelines inRCC renal cell carcinomaaccordance with the "Guidance for preparing treatment guidelines"published by MindsTSC =tuberous sclerosis(2020 version).The "Introduction"consisted of four sections,"Background Questionscomplex(BQ)"consisted of four sections,"Clinical Questions (CQ)"consisted of three sections,and "Future Questions (FQ)"consisted of three sections (total:14 sections).ConcerningCorrespondenceCQ,an agreement was confirmed through voting by the committee members based onNobuo Shinohara M.D..Ph.D.,the direction and strength of recommendation,accuracy of evidence,andDepartment of Renal andrecommendation comments.The present guidelines were updated based on the currentGenitourinary Surgery,evidence.We hope that the guidelines will provide guiding principles for the