We present a series of a distinct tumorous entity named renal angiomyoadenomatous tumor (RAT). Five cases were retrieved from the consultation files of the authors. Histologic and immunohistochemical features were evaluated. Sequencing analysis of coding region of the VHL gene was carried out in all cases. The tumors were composed of admixture of an epithelial clear cell component and prominent leiomyomatous stroma. Epithelial cells formed adenomatous tubular formations endowed with blister-like apical snouts. All tubular/glandular structures were lined by a fine capillary network. The epithelial component was positive for epithelial membrane antigen, CK7, CK20, AE1-AE3, CAM5.2, and vimentin in all cases. In all analyzed samples, no mutation of the VHL gene was found. RAT is a distinct morphologic entity, being different morphologically, immunohistochemically, and genetically from all renal tumors including conventional clear cell carcinoma and mixed epithelial and stromal tumor of kidney.

• MeSH
• Adenoma genetics   metabolism   pathology   MeSH
• Biomarkers metabolism   MeSH
• Epithelial Cells pathology   MeSH
• Keratin-20 metabolism   MeSH
• Keratin-7 metabolism   MeSH
• Keratins metabolism   MeSH
• Middle Aged MeSH
• Humans MeSH
• Mucin-1 metabolism   MeSH
• Mutation MeSH
• Von Hippel-Lindau Tumor Suppressor Protein genetics   MeSH
• Kidney Neoplasms genetics   metabolism   pathology   MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Vimentin metabolism   MeSH
• Loss of Heterozygosity MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH
• Research Support, Non-U.S. Gov't MeSH
• Names of Substances
• Biomarkers MeSH
• CAM 5.2 antigen MeSH Browser
• Keratin-20 MeSH
• Keratin-7 MeSH
• Keratins MeSH
• Mucin-1 MeSH
• Von Hippel-Lindau Tumor Suppressor Protein MeSH
• VHL protein, human MeSH Browser
• Vimentin MeSH