Malignant monotypic epithelioid angiomyolipoma of the kidney

Oxford University Hospitals NHS Trust, Oxford, England, United Kingdom
Clinical Radiology (Impact Factor: 1.76). 10/2004; 59(9):849-52. DOI: 10.1016/j.crad.2004.02.009
Source: PubMed


In summary, this case highlights the importance of considering epithelioid AML in the differential diagnosis of renal tumours, particularly in patients with associated conventional AMLs and/or other evidence of TS. In a patient with TS, a non-cystic renal mass is most likely to be an AML, even in the absence of radiological detection of fat due to haemorrhage or minimal fat content in an AML composed predominantly of smooth muscle. It may, however, represent a RCC or potentially a malignant epithelioid AML. Definitive demonstration of the benign nature of this patient's liver lesions was crucial for further management. © 2004 The Royal College of Radiologist. Published by Elsevier Ltd. All rights reserved.

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    • "There are no specific features of EAML in terms of clinical manifestations and imaging modalities. The appearance of the lesions is similar to the findings in patients with RCC [10]. Moreover, microscopically, the presence of epithelioid cells can result in difficulty in differentiating this tumor from RCC. "
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    ABSTRACT: Angiomyolipoma (AML) is a rare tumor mainly arising in the kidney. Here we report the case of a 55-year-old woman with malignant epithelioid angiomyolipoma with p53 gene mutation. After 7 years from radical nephrectomy of the left kidney, the patient developed multiple lung metastases that showed morphologic features overlapping those of the previously lesion, which was misdiagnosed as renal cell carcinoma. Both renal and pulmonary tumors were reevaluated by immunohistochemical assay, which were showed positive for HMB-45 and p53 protein (95%), but negative for epithelial markers and S-100 protein. A correct diagnosis of malignant epithelioid angiomyolipoma was made on the basis of those results. Meanwhile exon 8 mutation of p53 gene was detected in the renal tumor by microdissectionPCR-SSCP and sequencing technique indicating that p53 gene mutation may play an important role in malignant transformation. The patient was died of respiratory failure after 15 years' follow-up. This is the second report of renal malignant angiomyolipoma with p53 gene mutation.
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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
    Full-text · Article · Jan 2009
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    ABSTRACT: To study the radiological characteristics of renal masses in individuals with tuberous sclerosis complex (TSC) using serial CT, and to examine how renal cell carcinoma (RCC) may be differentiated from indeterminate cysts or masses. This was a retrospective study of 12 cases of TSC in which dedicated renal CT followed after US had demonstrated cystic or sonographically unusual renal masses. The CT density of all masses was measured and the masses categorized as simple cysts, complex cysts, angiomyolipomas or indeterminate solid masses. Subjects were maintained on regular follow-up with repeat CT or MRI and interval renal US. Indeterminate masses that showed rapid growth were considered suspicious for renal cell carcinoma and biopsy or nephrectomy followed. Comparative data were available for a median of 4 years. In each case the renal masses were multiple and bilateral; mean mass diameter was 3.6 cm. Among a total of 206 masses, 18 were simple cysts and 3 were complex cysts. Of the complex cysts, 1 proved to be an angiomyolipoma on histology and the other 2 showed no growth. Of the solid masses, 133 were typical angiomyolipomas (AMLs) and 52 were indeterminate. On follow-up, only 3 indeterminate masses showed rapid growth (>0.5 cm/year), of which only 1 proved to be an RCC on biopsy. The other 2 were minimal-fat AMLs, and the remainder of the masses showed no or slow growth. Many renal masses associated with TSC are radiologically indeterminate. A growth threshold of >0.5 cm/year identified the only RCC in this study (0.5% of all masses). Yearly radiological follow-up of indeterminate renal masses is recommended for individuals with TSC.
    No preview · Article · Jun 2005 · Clinical Radiology
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