Expression of CK7 and parvalbumin along with the absence of S100A1 is characteristic of chromophobe renal cell carcinoma, whereas strong and diffuse labeling of S100A1 along with the under-expression of CK7 and parvalbumin are typical of TFE3/TFEB-rearranged renal cell carcinoma. The gene discussed is S100A1; the disease is hereditary clear cell renal cell carcinoma.