Angiomyolipoma is the most common benign solid renal neoplasm observed in clinical practice. Once thought to be a hamartoma and almost always diagnosed by the imaged-based detection of fat, angiomyolipomas are now known to consist of a heterogeneous group of neoplasms. Although all are considered perivascular epithelioid cell tumors, many display different pathology, imaging features, and clinical behavior. The importance of understanding this group of neoplasms is emphasized by the fact that many types of angiomyolipoma contain little to no fat, and despite being benign, sometimes escape a pre-operative diagnosis. These types of angiomyolipomas can all be considered when encountering a renal mass that is both hyperattenuating relative to renal parenchyma on unenhanced CT and T2-hypointense, features that reflect their predominant smooth muscle component. We review recent developments and provide a radiological classification of angiomyolipomas that helps physicians understand the various types and learn how to both diagnose and manage them.

With the increasing discovery of small renal masses with cross-sectional imaging, there has been the concomitant rise in their treatment. With the intent of early curative surgery for a presumed renal cell carcinoma, many renal masses are being resected at surgery without a confirmed diagnosis. Many of them are benign, and some are angiomyolipomas. The diagnosis of renal angiomyolipoma using imaging is, therefore, is as important as ever. Although most, if not all angiomyolipomas with abundant fat are diagnosed readily, some have too little fat to be detected with imaging. This article reviews the current classification, imaging pitfalls, and diagnosis of angiomyolipoma with an emphasis on the fat-poor types. Proper imaging technique, a thorough search for fat, and the appropriate use of percutanoeus biopsy are all needed to eliminate the unnecessary treatment of these benign neoplasms.

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