Our results showed that both L1CAM and LINC01187 expression can help discern HOT from other renal cell carcinomas, especially the oncocytic renal tumor subtypes with overlapping morphologies; the unique checkered staining pattern exhibited by these markers in a mutually exclusive fashion, rather than the signal intensity, is a novel and distinct biomarker expression seen in HOT from BHD patients. Here, L1CAM is linked to Birt-Hogg-Dube syndrome.