ReviewPediatric nephrology (Berlin, Germany)2017
Treatment of renal angiomyolipoma in tuberous sclerosis complex (TSC) patients.
Review in Pediatric nephrology (Berlin, Germany), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 33 citations in OpenAlex.
- Trial
- Sirolimus application in patients with autoimmune-associated kidney diseases based on mTORC1 activation: a preliminary exploration and future perspectives.Frontiers in pharmacology · 2026Article
- Kidneys in Children with Tuberous Sclerosis Complex-An Up-to-Date Review.Journal of clinical medicine · 2025Review
- Comparison of perioperative efficacy and indications between laparoscopic capsule-preserving resection and partial nephrectomy for renal angiomyolipoma: a decade-long retrospective study.World journal of surgical oncology · 2025Article
- Synchronous angiomyolipoma with epithelial cysts and clear cell renal cell carcinoma: a case report.Frontiers in oncology · 2025Article
- Spontaneous angiomyolipoma rupture: A case of hemorrhagic shock and urgent embolization.Radiology case reports · 2024Article
- A case of tuberous sclerosis complex revealed by epilepsy.Radiology case reports · 2024Article
- Heterozygous germline FLCN mutation in Birt-Hogg-Dubé syndrome with bilateral renal hybrid oncocytic/chromophobe tumor and unilateral renal chromophobe cell carcinoma: a case report.Journal of cancer research and clinical oncology · 2023Article
- New Trends and Evidence for the Management of Renal Angiomyolipoma: A Comprehensive Narrative Review of the Literature.Journal of kidney cancer and VHL · 2022Review
- Chemical Biology Screening Identifies a Vulnerability to Checkpoint Kinase Inhibitors in TSC2-Deficient Renal Angiomyolipomas.Frontiers in oncology · 2022Article
- Genitourinary manifestations of hereditary cancer predisposition syndromes in children.Translational andrology and urology · 2020Review
- Therapeutic effects of the novel Leucyl-tRNA synthetase inhibitor BC-LI-0186 in non-small cell lung cancer.Therapeutic advances in medical oncology · 2019Article
- Sclerotic bone lesions as a potential imaging biomarker for the diagnosis of tuberous sclerosis complex.Scientific reports · 2018Article
- Efficacy and safety of Everolimus in children with TSC - associated epilepsy - Pilot data from an open single-center prospective study.Orphanet journal of rare diseases · 2016Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In adult tuberous sclerosis complex (TSC) patients, renal complications are the leading cause of death. Beginning in childhood, up to 80 % of patients develop renal angiomyolipoma characterized by a size-dependent risk of life-threatening bleeding. After discovery of the two causative genes, TSC1 and TSC2, and the role of mammalian target of rapamycin (mTOR) regulation in the pathogenesis of TSC, an increasing number of clinical studies evaluating mTOR inhibition in TSC patients have shown impressive results in many organ manifestations, such as brain, lung, and kidney. For renal angiomyolipoma, mTOR inhibitor treatment fundamentally changed the approach from preventive embolization or even partial nephrectomy to everolimus treatment in order to preserve kidney function.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.