Clear-cell papillary renal cell carcinoma: 24 cases of a distinct low-grade renal tumor and a comparative genomic hybridization array study of 7 cases.
Résumé
Aim: To report clinicopathological and genomic characteristics of clear-cell papillary renal carcinoma (ccpRCC), a rare, recently characterized renal tumor entity. Methods and results: Twenty-four renal tumors identified as ccpRCC were gathered. Data from comparative genomic hybridization on microarrays (array-CGH) were obtained for 7 of them. Most tumors (58%) occurred in the absence of renal disease. Mean patient age was 58.1 years. Tumors were small (mean size: 2.4 cm) and classified as pT1a. Histological characteristics consisted of tubes and papillae lined by a single layer of small clear cells harboring low-grade nuclei (Fuhrman grade 1 or 2). Architectural variations, with compact areas (41% of cases) and a micro- or macrocystic pattern (67% of cases) were frequently observed. Immunostaining demonstrated diffuse, strong expression of cytokeratin 7 and vimentin, whereas CD10, racemase, RCC antigen, TFE3 and TFEB were consistently negative. In 7 tumors, array-CGH detected no chromosome imbalances. Conclusion: ccpRCC were differentiated from other renal neoplasms by the specific association of histopathological and immunohistochemical features, without characteristic genomic imbalances. Clinical, histopathological and genomic data suggested that this tumor have low potential for malignancy.
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