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CUAJ – CUA Best Practice Report                                                          Guo et al
                                                                                    BPL: AML management



            previously thought. Other factors that may influence the desire to treat include access to health
            care, women of childbearing age, and patient preferences.

            Up to 92% of AMLs in contemporary series are asymptomatic; however, when symptoms are
            present, treatment should be considered to improve symptoms. Symptoms such as flank pain, palpable
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            mass or gross hematuria are more likely in larger lesions.  Based on natural history and the minimal risk
            of hemorrhage, small AML (<4cm) rarely require intervention.
                   For AMLs larger than 4 cm, treatment should be discussed. While they do appear to be at a
            higher risk, the absolute risk of spontaneous hemorrhage seems lower than previously estimated, and
            there is limited evidence for an absolute size threshold. In addition to size, several other factors may
            play a role in assessing the risk of hemorrhage of untreated AML. The presence of aneurysms and
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            aneurysmal size has been linked to the risk of hemorrhage in several studies.  However, intratumoural
            aneurysms can only be reliably assessed through angiography and may not be clinically feasible for the
            majority of cases. 38,39
                   Ongoing surveillance is a necessary pillar of AML management. For patients who have poor
            access to imaging or emergency care treatment, or who do not desire long term monitoring,
            consideration (weighing risks/benefits) may be given to intervention.
                   Finally, hemorrhage of AML during pregnancy is an uncommon yet greatly feared complication.
            There may be a physiologic basis to this increased risk with estrogen receptor expression strongly
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            associated with AMLs.  The only clinical evidence we have to rely upon are case reports. Cetin et al.
            reviewed 26 case reports of AML during pregnancy in literature from 1994 to 2015 and found 81%
            presented with rupture (mean size 11cm). Current evidence for rupture in this population is extremely
            weak and is based on case reports and a physiologic hypothesis. 41,42  However, given the high trade-offs,
            the treatment of AMLs should be considered and discussed with reproductive-age women. These
            recommendations are consistent with the most recent EAU RCC guidelines.

            Interventions

            Acutely bleeding AMLs


            Recommendation #5: Transcatheter embolization should be the first-line treatment for acutely
            bleeding AML.

            There have not been any prospective trials comparing interventions in acutely hemorrhaging AMLs.
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            Traditionally, selective TAE has been the first-line treatment.  Compared to surgery, TAE is minimally
            invasive and preserves renal function compared to surgery, especially given the concern for the
            requirement of radical nephrectomy in this setting.
                   While this is minimal data in the acute setting, in general, embolization does appear to be
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            associated with fewer complications but may have an increased risk of repeat intervention.  A surgical
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