Renal angiomyolipoma (AML) may present as rare variants such as epithelioid and AML with epithelial cysts posing difficulties for the diagnosis to the surgical pathologist. We report a case of a 46-year-old male patient presenting a 5-cm solid tumor in the lower pole of the left kidney, with cystic changes at cut surface. The tumor exhibited 95% of epithelioid cells with atypical nuclei. A small focus of typical AML was observed. The immunoprofile of tumor cells was classical of AML including expression of melanocytic markers such as HMB45 and Melan A. We report the immunohistochemical study of the cystic component in an epithelioid AML. In contrast to the immunoreactivity reported in typical AML, the present case shows obvious expression of melanocytic markers in the cystic epithelial lining. This is strong evidence that these cysts are neoplastic and derived from AML, rather than entrapped native collecting duct epithelium.
The data presented herein suggest the occurrence of changes in the distribution of nephrological diseases in Salvador, Brazil. The disease that was most prevalent shifted from membranoproliferative glomerulonephritis to focal and segmental glomerulosclerosis from 1975 to 2006 and from focal and segmental glomerulosclerosis to lupus nephritis from 2006 to 2015.
Chronic lesions in renal biopsies are a well recognized prognostic factor for renal diseases, including lupus nephritis. The methods used for assessment of chronic lesions are, however, largely based on semiquantitative evaluation and may lead to poor reproducibility. Interobserver variation is particularly important in lupus nephritis, in which acute and chronic lesions may occur simultaneously. In this study we tested the reproducibility of chronic lesion assessment performed by three pathologists, two with specific training in renal pathology, using 20 renal biopsies and a standard semiquantitative method. In a second experiment, we evaluated the reproducibility of chronic lesion assessment in 33 biopsies of lupus nephritis by the two nephropathologists. The semiquantitative estimated values were compared with those from a previously proposed morphometric method for quantification of chronic lesions in renal biopsies. Although correlations were observed among the estimated values, there was a wide range of variation when semiquantitative methods were used. In particular, activity and chronicity indices of lupus nephritis were poorly reproducible. In contrast, use of a morphometric score, although not eliminating interobserver variability, led to better reproducibility of estimated values than that obtained with semiquantitative methods.
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