CONTEXT: Little is known about the natural history of sporadic angiomyolipomas (AMLs); there is uncertainty regarding the indications of treatment and treatment options. OBJECTIVE: To evaluate the indications, effectiveness, harms, and follow-up of different management modalities for sporadic AML to provide guidance for clinical practice. EVIDENCE ACQUISITION: A systematic review of the literature was undertaken, incorporating Medline, Embase, and the Cochrane Library (from 1 January 1990 to 30 June 2017), in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. No restriction on study design was imposed. Patients with sporadic AML were included. The main interventions included active surveillance, surgery (nephron-sparing surgery and radical nephrectomy), selective arterial embolisation, and percutaneous or laparoscopic thermal ablations (radiofrequency, microwaves, or cryoablation). The outcomes included indications for active treatment, AML growth rate, AML recurrence rate, risk of bleeding, post-treatment renal function, adverse events of treatments, and modalities of follow-up. Risk of bias assessment was performed using standard Cochrane methods. EVIDENCE SYNTHESIS: Among 2704 articles identified, 43 were eligible for inclusion (zero randomised controlled trials, nine nonrandomised comparative retrospective studies, and 34 single-arm case series). Most studies were retrospective and uncontrolled, and had a moderate to high risk of bias. CONCLUSIONS: In active surveillance series, spontaneous bleeding was reported in 2% of patients and active treatment was undertaken in 5%. Active surveillance is the most chosen option in 48% of the cases, followed by surgery in 31% and selective arterial embolisation in 17% of the cases. Selective arterial embolisation appeared to reduce AML volume but required secondary treatment in 30% of the cases. Surgery (particularly nephron-sparing surgery) was the most effective treatment in terms of recurrence and need for secondary procedures. Thermal ablation was an infrequent option. The association between AML size and the risk of bleeding remained unclear; as such the traditional 4-cm cut-off should not per se trigger active treatment. In spite of the limitations and uncertainties relating to the evidence base, the findings may be used to guide and inform clinical practice, until more robust data emerge. PATIENT SUMMARY: Sporadic angiomyolipoma (AML) is a benign tumour of the kidney consisting of a mixture of blood vessels, fat, and muscle. Large tumours may have a risk of spontaneous bleeding. However, the size beyond which these tumours need to be treated remains unclear. Most small AMLs can be monitored without any active treatment. For those who need treatment, options include surgical removal of the tumour or stopping its blood supply (selective embolisation). Surgery has a lower recurrence rate and lower need for a repeat surgical procedure.

Academic Urology Unit University of Aberdeen Aberdeen UK

Department of Cancer Medicine Gustave Roussy Université Paris Saclay Villejuif France

Department of Clinical Sciences Lund Skåne University Hospital Lund Sweden

Department of Nephrology and Hypertension Patient Advocate International Kidney Cancer Coalition University Medical Center Utrecht Utrecht The Netherlands

Department of Surgical and Perioperative Sciences Urology and Andrology Umeå University Umeå Sweden

Department of Urology and Urologic Oncology Hannover Medical School Hannover Germany

Department of Urology Cabueñes University Hospital Gijón Spain

Department of Urology Chaim Sheba Medical Center Tel Hashomer Ramat Gan Israel

Department of Urology Coimbra University Hospital Coimbra Portugal

Department of Urology Ludwig Maximilians University Munich Germany

Department of Urology Royal Free Hospital Pond Street London UK

Department of Urology Sunderby Hospital Sunderby Sweden

Department of Urology University Hospital Hamburg Eppendorf Hamburg Germany

Department of Urology University Hospital Plzen and Faculty of Medicine in Plzen Charles Universtity Czech Republic

Department of Urology University Hospital Schleswig Holstein Lübeck Germany

Department of Urology University of Rennes Rennes France

Division of Urology Maggiore della Carità Hospital University of Eastern Piedmont Novara Italy

The Royal Free London NHS Foundation Trust and UCL Division of Surgery and Interventional Science London UK; Department of Urology The Netherlands Cancer Institute Antoni van Leeuwenhoek Hospital Amsterdam The Netherlands

The Royal Free NHS Trust and Barts Cancer Institute Queen Mary University of London London UK

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