{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T20:12:33Z","timestamp":1762200753757,"version":"build-2065373602"},"reference-count":80,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T00:00:00Z","timestamp":1762128000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Tuberous sclerosis complex (TSC) is a rare genetic disorder characterized by the growth of benign tumors in various organ systems, with particularly significant effects on the kidneys. Renal manifestations of TSC include angiomyolipomas (AMLs), renal cysts, and a higher risk of renal cell carcinoma (RCC). Nephrological monitoring is crucial for the early detection of kidney changes, the management of hypertension, and the assessment of the risk of developing chronic kidney disease. Ultrasound is typically the initial imaging choice for diagnosis and monitoring, with magnetic resonance imaging (MRI) being a preferred imaging modality for long-term surveillance. Patients with TSC have an increased risk of arterial hypertension, renal artery stenosis, and urolithiasis. In some patients, the co-occurrence of TSC and autosomal dominant polycystic kidney disease (ADPKD) is caused by the TSC2\/PKD1 contiguous gene syndrome (CGS). The primary medical treatment for TSC is a mammalian target of rapamycin kinase inhibitors (mTOR), as they effectively shrink tumors, often reducing or eliminating the need for surgical intervention. Methods: This article aims to review the most recent literature on the diagnosis and management of renal lesions in tuberous sclerosis complex (TSC), with a particular focus on the role of various imaging techniques. Conclusions: Given the multifactorial nature of this disease, this review emphasizes the importance of a multidisciplinary approach, including various imaging methods, to improve the care and treatment outcomes of children with tuberous sclerosis complex.<\/jats:p>","DOI":"10.3390\/jcm14217805","type":"journal-article","created":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T19:30:27Z","timestamp":1762198227000},"page":"7805","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Kidneys in Children with Tuberous Sclerosis Complex\u2014An Up-to-Date Review"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-3339-8157","authenticated-orcid":false,"given":"Anna Maria","family":"Wabik","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Nephrology, Medical University of Warsaw, 02-091 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9238-0391","authenticated-orcid":false,"given":"Jakub","family":"Pytlos","sequence":"additional","affiliation":[{"name":"Department of Pediatric Radiology, Medical University of Warsaw, 02-091 Warsaw, Poland"},{"name":"2nd Department of Clinical Radiology, Doctoral School, Medical University of Warsaw, 02-091 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-1353-2575","authenticated-orcid":false,"given":"Aneta","family":"Michalczewska","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Nephrology, Medical University of Warsaw, 02-091 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1959-8255","authenticated-orcid":false,"given":"Piotr","family":"Skrzypczyk","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Nephrology, Medical University of Warsaw, 02-091 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,3]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1186\/s13023-018-0870-y","article-title":"Incidence of Tuberous Sclerosis and Age at First Diagnosis: New Data and Emerging Trends from a National, Prospective Surveillance Study","volume":"13","author":"Mann","year":"2018","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1186\/s13023-020-01517-2","article-title":"Prevalence, Mortality and Healthcare Economic Burden of Tuberous Sclerosis in Hong Kong: A Population-Based Retrospective Cohort Study (1995\u20132018)","volume":"15","author":"Chu","year":"2020","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1001\/archneur.57.5.662","article-title":"National Institutes of Health Consensus Conference: Tuberous Sclerosis Complex","volume":"57","author":"Hyman","year":"2000","journal-title":"Arch. Neurol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1186\/s13023-022-02266-0","article-title":"Perfect Match: MTOR Inhibitors and Tuberous Sclerosis Complex","volume":"17","author":"Luo","year":"2022","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_5","first-page":"134","article-title":"The Recommendations for the Management of Tuberous Sclerosis Complex Patients with Kidney Manifestation. The Consensus Statement of Tuberous Sclerosis Complex Work Group of Polish Society of Nephrology","volume":"20","author":"Tarasewicz","year":"2016","journal-title":"Nefrol. Dial. Pol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.pediatrneurol.2021.07.011","article-title":"Updated International Tuberous Sclerosis Complex Diagnostic Criteria and Surveillance and Management Recommendations","volume":"123","author":"Northrup","year":"2021","journal-title":"Pediatr. Neurol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and Guidelines for the Interpretation of Sequence Variants: A Joint Consensus Recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology","volume":"17","author":"Richards","year":"2015","journal-title":"Genet. Med."},{"key":"ref_8","unstructured":"Northrup, H., Koenig, M.K., Pearson, D.A., and Au, K.S. (1993). Tuberous Sclerosis Complex, Oxford Academic."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1590\/0100-3984.2016.0020","article-title":"Imaging of Tuberous Sclerosis Complex: A Pictorial Review","volume":"50","author":"Faria","year":"2017","journal-title":"Radiol. Bras."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1007\/s10689-017-0029-3","article-title":"Pancreatic Neuroendocrine Tumor in a Patient with a TSC1 Variant: Case Report and Review of the Literature","volume":"17","author":"Mortaji","year":"2018","journal-title":"Fam. Cancer"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.acra.2020.12.019","article-title":"Tuberous Sclerosis Complex (TSC): Renal and Extrarenal Imaging","volume":"29","author":"Gupta","year":"2022","journal-title":"Acad. Radiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2927","DOI":"10.1007\/s00467-024-06417-2","article-title":"The Extent of Kidney Involvement in Paediatric Tuberous Sclerosis Complex","volume":"39","author":"Limavady","year":"2024","journal-title":"Pediatr. Nephrol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1615\/CritRevOncog.2022042857","article-title":"Progress in Tuberous Sclerosis Complex Renal Disease","volume":"27","author":"Bissler","year":"2022","journal-title":"Crit. Rev. Oncog."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1007\/s00467-020-04775-1","article-title":"Renal Tumors in Tuberous Sclerosis Complex","volume":"36","author":"Trnka","year":"2021","journal-title":"Pediatr. Nephrol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"41","DOI":"10.5334\/jbsr.1536","article-title":"Renal Angiomyolipoma: The Good, the Bad, and the Ugly","volume":"102","author":"Vos","year":"2018","journal-title":"J. Belg. Soc. Radiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/S0022-5347(01)63072-6","article-title":"Renal Lesion Growth in Children with Tuberous Sclerosis Complex","volume":"160","author":"Ewalt","year":"1998","journal-title":"J. Urol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1097\/PAS.0b013e31817ed7a6","article-title":"Renal Angiomyolipoma","volume":"33","author":"Aydin","year":"2009","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1378\/chest.117.1.25","article-title":"Frequent Estrogen and Progesterone Receptor Immunoreactivity in Renal Angiomyolipomas From Women With Pulmonary Lymphangioleiomyomatosis","volume":"117","author":"Logginidou","year":"2000","journal-title":"Chest"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1111\/j.1523-1755.2004.00838.x","article-title":"Renal Angiomyolipomata","volume":"66","author":"Bissler","year":"2004","journal-title":"Kidney Int."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"826","DOI":"10.2214\/AJR.17.17973","article-title":"Renal Angiomyolipoma: Radiologic Classification and Imaging Features According to the Amount of Fat","volume":"209","author":"Park","year":"2017","journal-title":"Am. J. Roentgenol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"5","DOI":"10.15586\/jkcvhl.2020.131","article-title":"Renal Manifestations of Tuberous Sclerosis Complex","volume":"7","author":"Nair","year":"2020","journal-title":"J. Kidney Cancer"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1002\/ajmg.c.31654","article-title":"Renal Manifestation of Tuberous Sclerosis Complex","volume":"178","author":"Bissler","year":"2018","journal-title":"Am. J. Med. Genet. C Semin. Med. Genet."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1016\/S0092-8674(00)81793-6","article-title":"The Molecular Basis of Focal Cyst Formation in Human Autosomal Dominant Polycystic Kidney Disease Type I","volume":"87","author":"Qian","year":"1996","journal-title":"Cell"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"3253","DOI":"10.1007\/s00467-022-05820-x","article-title":"Cystic Kidney Disease in Tuberous Sclerosis Complex: Current Knowledge and Unresolved Questions","volume":"38","author":"Kilcoyne","year":"2023","journal-title":"Pediatr. Nephrol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1038\/s41581-018-0059-6","article-title":"Renal Disease in Tuberous Sclerosis Complex: Pathogenesis and Therapy","volume":"14","author":"Lam","year":"2018","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"bcr2013010015","DOI":"10.1136\/bcr-2013-010015","article-title":"Bilateral Renal Cell Carcinoma in a Paediatric Patient with Tuberous Sclerosis Complex","volume":"2013","author":"Gil","year":"2013","journal-title":"BMJ Case Rep."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1148\/radiographics.19.5.g99se231388","article-title":"Pediatric Case of the Day","volume":"19","author":"Allison","year":"1999","journal-title":"RadioGraphics"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1097\/PAS.0000000000000248","article-title":"Tuberous Sclerosis\u2013Associated Renal Cell Carcinoma","volume":"38","author":"Guo","year":"2014","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_29","doi-asserted-by":"crossref","unstructured":"Henske, E.P., Cornejo, K.M., and Wu, C.-L. (2021). Renal Cell Carcinoma in Tuberous Sclerosis Complex. Genes, 12.","DOI":"10.3390\/genes12101585"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1148\/radiol.2018181243","article-title":"Imaging of Kidney Cysts and Cystic Kidney Diseases in Children: An International Working Group Consensus Statement","volume":"290","author":"Gimpel","year":"2019","journal-title":"Radiology"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1148\/radiology.158.1.3510019","article-title":"The Current Radiological Approach to Renal Cysts","volume":"158","author":"Bosniak","year":"1986","journal-title":"Radiology"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1148\/radiol.2019182646","article-title":"Bosniak Classification of Cystic Renal Masses, Version 2019: An Update Proposal and Needs Assessment","volume":"292","author":"Silverman","year":"2019","journal-title":"Radiology"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"2109","DOI":"10.1097\/HJH.0000000000001423","article-title":"Diagnosis of Tuberous Sclerosis Complex in a Patient Referred for Uncontrolled Hypertension and Renal Dysfunction","volume":"35","author":"Sarafidis","year":"2017","journal-title":"J. Hypertens."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1116","DOI":"10.1080\/13696998.2016.1202254","article-title":"The Economic Burden of Tuberous Sclerosis Complex in UK Patients with Renal Manifestations: A Retrospective Cohort Study in the Clinical Practice Research Datalink (CPRD)","volume":"19","author":"Kingswood","year":"2016","journal-title":"J. Med. Econ."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"2085","DOI":"10.1007\/s00467-018-4003-6","article-title":"Renal Progression Factors in Young Patients with Tuberous Sclerosis Complex: A Retrospective Cohort Study","volume":"33","author":"Janssens","year":"2018","journal-title":"Pediatr. Nephrol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1089\/cap.2016.0081","article-title":"The Treatment of Severe Childhood Aggression Study: 12 Weeks of Extended, Blinded Treatment in Clinical Responders","volume":"27","author":"Findling","year":"2017","journal-title":"J. Child. Adolesc. Psychopharmacol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1038\/nrneph.2012.19","article-title":"Glomerular Hyperfiltration: Definitions, Mechanisms and Clinical Implications","volume":"8","author":"Helal","year":"2012","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1159\/000448293","article-title":"Review of the Tuberous Sclerosis Renal Guidelines from the 2012 Consensus Conference: Current Data and Future Study","volume":"134","author":"Kingswood","year":"2016","journal-title":"Nephron"},{"key":"ref_39","first-page":"e67800","article-title":"Progressive Polycystic Kidney Disease in an Infant Girl With TSC2\/PKD1 Contiguous Gene Syndrome","volume":"16","author":"Hashimoto","year":"2024","journal-title":"Cureus"},{"key":"ref_40","doi-asserted-by":"crossref","unstructured":"Nass, R.D., Hampel, K.G., Elger, C.E., and Surges, R. (2019). Blood Pressure in Seizures and Epilepsy. Front. Neurol., 10.","DOI":"10.3389\/fneur.2019.00501"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"e20171904","DOI":"10.1542\/peds.2017-1904","article-title":"Clinical Practice Guideline for Screening and Management of High Blood Pressure in Children and Adolescents","volume":"140","author":"Flynn","year":"2017","journal-title":"Pediatrics"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1887","DOI":"10.1097\/HJH.0000000000001039","article-title":"2016 European Society of Hypertension Guidelines for the Management of High Blood Pressure in Children and Adolescents","volume":"34","author":"Lurbe","year":"2016","journal-title":"J. Hypertens."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1016\/j.kint.2020.10.026","article-title":"Executive Summary of the KDIGO 2021 Clinical Practice Guideline for the Management of Blood Pressure in Chronic Kidney Disease","volume":"99","author":"Cheung","year":"2021","journal-title":"Kidney Int."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1038\/s41581-024-00818-0","article-title":"Clinical Practice Recommendations for Kidney Involvement in Tuberous Sclerosis Complex: A Consensus Statement by the ERKNet Working Group for Autosomal Dominant Structural Kidney Disorders and the ERA Genes & Kidney Working Group","volume":"20","author":"Mekahli","year":"2024","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1007\/s00467-010-1466-5","article-title":"Vascular Involvement in Tuberous Sclerosis","volume":"25","author":"Salerno","year":"2010","journal-title":"Pediatr. Nephrol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"233","DOI":"10.2147\/TCRM.S348366","article-title":"Diagnosis and Management of Genetic Causes of Middle Aortic Syndrome in Children: A Comprehensive Literature Review","volume":"18","author":"Lazea","year":"2022","journal-title":"Ther. Clin. Risk Manag."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1093\/ckj\/sfx094","article-title":"Recommendations for Imaging-Based Diagnosis and Management of Renal Angiomyolipoma Associated with Tuberous Sclerosis Complex","volume":"10","author":"Torra","year":"2017","journal-title":"Clin. Kidney J."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1053\/j.sult.2016.11.001","article-title":"Diagnosis of Renal Angiomyolipomas: Classic, Fat-Poor, and Epithelioid Types","volume":"38","author":"Jinzaki","year":"2017","journal-title":"Semin. Ultrasound CT MRI"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1016\/j.crad.2005.01.009","article-title":"Tuberose Sclerosis Complex: Analysis of Growth Rates Aids Differentiation of Renal Cell Carcinoma from Atypical or Minimal-Fat-Containing Angiomyolipoma","volume":"60","author":"Patel","year":"2005","journal-title":"Clin. Radiol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.crad.2009.09.014","article-title":"The Radiological Diagnosis and Treatment of Renal Angiomyolipoma\u2014Current Status","volume":"65","author":"Halpenny","year":"2010","journal-title":"Clin. Radiol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1016\/j.ejpn.2015.11.011","article-title":"The Clinical Profile of Tuberous Sclerosis Complex (TSC) in the United Kingdom: A Retrospective Cohort Study in the Clinical Practice Research Datalink (CPRD)","volume":"20","author":"Kingswood","year":"2016","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1868","DOI":"10.2214\/ajr.184.6.01841868","article-title":"The Use of Opposed-Phase Chemical Shift MRI in the Diagnosis of Renal Angiomyolipomas","volume":"184","author":"Israel","year":"2005","journal-title":"Am. J. Roentgenol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1007\/s00261-014-0083-3","article-title":"Renal Angiomyolipoma: A Radiological Classification and Update on Recent Developments in Diagnosis and Management","volume":"39","author":"Jinzaki","year":"2014","journal-title":"Abdom. Imaging"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1148\/radiol.12112087","article-title":"Angiomyolipoma with Minimal Fat: Can It Be Differentiated from Clear Cell Renal Cell Carcinoma by Using Standard MR Techniques?","volume":"265","author":"Hindman","year":"2012","journal-title":"Radiology"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1148\/radiol.211680","article-title":"Multicenter Evaluation of Multiparametric MRI Clear Cell Likelihood Scores in Solid Indeterminate Small Renal Masses","volume":"303","author":"Schieda","year":"2022","journal-title":"Radiology"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"W178","DOI":"10.2214\/AJR.12.8776","article-title":"Renal Epithelioid Angiomyolipoma: Imaging Characteristics in Nine Cases With Radiologic-Pathologic Correlation and Review of the Literature","volume":"200","author":"Froemming","year":"2013","journal-title":"Am. J. Roentgenol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1097\/00004728-199309000-00015","article-title":"Improved CT Fat Detection in Small Kidney Angiomyolipomas Using Thin Sections and Single Voxel Measurements","volume":"17","author":"Kurosaki","year":"1993","journal-title":"J. Comput. Assist. Tomogr."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"769","DOI":"10.2214\/ajr.179.3.1790769","article-title":"CT Differentiation of Large Exophytic Renal Angiomyolipomas and Perirenal Liposarcomas","volume":"179","author":"Israel","year":"2002","journal-title":"Am. J. Roentgenol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1097\/RCT.0b013e3181507534","article-title":"Computed Tomographic Distinction of Perirenal Liposarcoma From Exophytic Angiomyolipoma","volume":"32","author":"Ellingson","year":"2008","journal-title":"J. Comput. Assist. Tomogr."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1836","DOI":"10.1093\/hmg\/ddu597","article-title":"A Shower of Second Hit Events as the Cause of Multifocal Renal Cell Carcinoma in Tuberous Sclerosis Complex","volume":"24","author":"Tyburczy","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"703","DOI":"10.2215\/CJN.07371009","article-title":"Increased Incidence of Angioedema with ACE Inhibitors in Combination with MTOR Inhibitors in Kidney Transplant Recipients","volume":"5","author":"Duerr","year":"2010","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1007\/s40620-020-00714-3","article-title":"From Tuberous Sclerosis Complex to End Stage Renal Disease: Who Are These Patients?","volume":"34","author":"Vabret","year":"2021","journal-title":"J. Nephrol."},{"key":"ref_63","first-page":"CD011272","article-title":"Rapamycin and Rapalogs for Tuberous Sclerosis Complex","volume":"2017","author":"Sasongko","year":"2016","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1016\/j.urology.2017.02.036","article-title":"Patterns of Disease Monitoring and Treatment Among Patients With Tuberous Sclerosis Complex-Related Angiomyolipomas","volume":"104","author":"Swallow","year":"2017","journal-title":"Urology"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1016\/j.jpeds.2017.05.015","article-title":"Improvement in Renal Cystic Disease of Tuberous Sclerosis Complex After Treatment with Mammalian Target of Rapamycin Inhibitor","volume":"187","author":"Siroky","year":"2017","journal-title":"J. Pediatr."},{"key":"ref_66","doi-asserted-by":"crossref","unstructured":"Franz, D.N., Belousova, E., Sparagana, S., Bebin, E.M., Frost, M.D., Kuperman, R., Witt, O., Kohrman, M.H., Flamini, J.R., and Wu, J.Y. (2016). Long-Term Use of Everolimus in Patients with Tuberous Sclerosis Complex: Final Results from the EXIST-1 Study. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0158476"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1016\/S0140-6736(12)61767-X","article-title":"Everolimus for Angiomyolipoma Associated with Tuberous Sclerosis Complex or Sporadic Lymphangioleiomyomatosis (EXIST-2): A Multicentre, Randomised, Double-Blind, Placebo-Controlled Trial","volume":"381","author":"Bissler","year":"2013","journal-title":"Lancet"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1093\/ndt\/gfv249","article-title":"Everolimus for Renal Angiomyolipoma in Patients with Tuberous Sclerosis Complex or Sporadic Lymphangioleiomyomatosis: Extension of a Randomized Controlled Trial","volume":"31","author":"Bissler","year":"2016","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1093\/ndt\/gfy132","article-title":"Effect of Everolimus on Renal Function in Patients with Tuberous Sclerosis Complex: Evidence from EXIST-1 and EXIST-2","volume":"34","author":"Bissler","year":"2019","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1186\/s13023-018-0781-y","article-title":"Assessing the Outcomes of Everolimus on Renal Angiomyolipoma Associated with Tuberous Sclerosis Complex in China: A Two Years Trial","volume":"13","author":"Cai","year":"2018","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_71","unstructured":"(2025, September 19). Pfizer Rapamune 1 Mg\/ML Oral Solution SmPC. Available online: https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/rapamune-epar-product-information_en.pdf."},{"key":"ref_72","unstructured":"(2025, September 19). Novartis Europharm Afinitor 2.5 Mg Tablets SmPC. Available online: https:\/\/www.ema.europa.eu\/pl\/documents\/product-information\/afinitor-epar-product-information_pl.pdf."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.1007\/s00467-016-3474-6","article-title":"Treatment of Renal Angiomyolipoma in Tuberous Sclerosis Complex (TSC) Patients","volume":"32","author":"Brakemeier","year":"2017","journal-title":"Pediatr. Nephrol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1114","DOI":"10.3109\/0886022X.2014.918784","article-title":"Emergency Embolization of Actively Bleeding Renal Angiomyolipoma in a Patient of Tuberous Sclerosis","volume":"36","author":"Parekh","year":"2014","journal-title":"Ren. Fail."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"1777","DOI":"10.1038\/sj.ki.5001853","article-title":"Renal Manifestations of Tuberous Sclerosis Complex: Incidence, Prognosis, and Predictive Factors","volume":"70","author":"Rakowski","year":"2006","journal-title":"Kidney Int."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1053\/j.ajkd.2005.09.028","article-title":"Embolization of Renal Angiomyolipomata in Patients With Tuberous Sclerosis Complex","volume":"47","author":"Williams","year":"2006","journal-title":"Am. J. Kidney Dis."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1764","DOI":"10.1097\/01.ju.0000177497.31986.64","article-title":"Long-Term Outcome of Transcatheter Embolization of Renal Angiomyolipomas Due to Tuberous Sclerosis Complex","volume":"174","author":"Ewalt","year":"2005","journal-title":"J. Urol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"48","DOI":"10.2215\/CJN.10250620","article-title":"Multicenter Study of Long-Term Safety of Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease","volume":"16","author":"Torres","year":"2021","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"100639","DOI":"10.1016\/j.xkme.2023.100639","article-title":"Tolvaptan and Kidney Function Decline in Older Individuals With Autosomal Dominant Polycystic Kidney Disease: A Pooled Analysis of Randomized Clinical Trials and Observational Studies","volume":"5","author":"Chebib","year":"2023","journal-title":"Kidney Med."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"9820181","DOI":"10.1155\/2017\/9820181","article-title":"Recent Advances and Challenges of MTOR Inhibitors Use in the Treatment of Patients with Tuberous Sclerosis Complex","volume":"2017","author":"Palavra","year":"2017","journal-title":"Oxid. Med. Cell. 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