Malignant monotypic epithelioid angiomyolipoma of the kidney

Oxford University Hospitals NHS Trust, Oxford, England, United Kingdom
Clinical Radiology (Impact Factor: 1.66). 10/2004; 59(9):849-52. DOI: 10.1016/j.crad.2004.02.009
Source: PubMed
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    ABSTRACT: To explore the characteristics of multi-slice computed tomography (CT) manifestations of hepatic epithelioid angiomyolipoma (HEA), improve the rate of accurate diagnosis, and reduce the misdiagnostic rate. The multi-slice CT manifestations in five patients who were diagnosed with HEA definitely by postoperative pathological examination were analysed retrospectively. Three female patients and two male patients were included. Before operation, four patients received plain CT scanning and dynamic enhancement scanning, and the other patient only received enhancement scanning, with immunohistochemical analysis conducted after postoperative pathological examination. Four patients were misdiagnosed by CT, including three patients misdiagnosed with hepatic cell carcinoma and one patient with focal nodular hyperplasia. Upper abdominal multi-slice spiral CT scanning and three-stage enhancement scanning were conducted in five patients with HEA before operation. HEA had certain characteristic CT manifestations: low density masses, a few relatively high-density masses or fat-density masses diffusely shown in foci, clear boundary, round or oval and large focus, and tumour size ranging from 3.1 cm × 2.5 cm to 7.0 cm × 5.2 cm. During enhancement scanning, the foci were significantly enhanced uniformly or non-uniformly during the arterial phase, while during the venous and equilibrium phases, the foci were enhanced continuously or showed obvious low-density masses. Obviously enhanced and widened vessels could be found adjacent to foci or in the central area of foci during the arterial phase. CT manifestations of HEA have certain characteristics. Primary diagnosis can be obtained by combining CT findings with clinical data, but pathological examination is still needed for a definite diagnosis.
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    ABSTRACT: The epithelioid variant of angiomyolipomas has been documented to have the ability to behave in an aggressive manner. We report a 38-year-old woman who presented with progressive left flank pain for several months. A renal angiomyolipoma with hemorrhage was diagnosed with an imaging study, and she underwent surgical removal due to persistent symptoms. Epithelioid features were observed in the histologic examination. Positive immunostaining for HMB-45 further supported the diagnosis. Although the postoperative period in this patient was uneventful after 9 months of follow-up, regular postoperative surveillance is significant in light of the malignant potential of epithelioid
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    ABSTRACT: 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.
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