Sporadic Renal Angiomyolipoma: Can We Adopt a Uniform Management Protocol?

Abstract

Renal angiomyolipomas (AMLs), formerly known as PEComas (tumors showing perivascular epithelioid cell differentiation) are common benign renal masses composed of a varying ratio of fat, blood vessels, and smooth muscles. They are largely asymptomatic and diagnosed incidentally on imaging.The adipose tissue content is the factor that gives AMLs their characteristic appearance on imaging and makes them easily identifiable. However, the fat-poor or fat-invisible varieties, which are difficult to differentiate radiologically from renal cell carcinomas (RCCs), present a diagnostic challenge. It is thus essential to establish the diagnosis and identify the atypical and hereditary cases as they require more intense surveillance and management due to their potential for malignant transformation.Multiple management options are available, ranging from conservative approach to embolization and to the more radical option of nephrectomy. While the indications for intervention are relatively clear and aimed at a rather small cohort, the protocol for follow-up of the remainder of the cohort forming the majority of cases is not well established. The surveillance and discharge policies therefore vary between institutions and even between individual practitioners. We have reviewed the literature to establish an optimum management pathway focusing on the typical AMLs.

Multiple renal angiomyolipomas with asymptomatic nontraumatic pulmonary fat embolus: A case report

“…4 The incidental detection of AML is common, and while mostly asymptomatic, a few cases may manifest symptoms such as pain or complications like spontaneous bleeding. 5 Renal AML does not extend beyond the kidney. Nevertheless, there are rare instances, where AML may manifest as pulmonary fat embolism.…”

“…Female predilection is apparent in sporadic cases, as evidenced by a female‐to‐male ratio of 4:1 4 . The incidental detection of AML is common, and while mostly asymptomatic, a few cases may manifest symptoms such as pain or complications like spontaneous bleeding 5 . Renal AML does not extend beyond the kidney.…”