PAX8 immunostaining and CD68 (PG-M1) negativity support the diagnosis of TFEB-rearranged renal cell carcinoma, whereas pure epithelioid PEComa/epithelioid angiomyolipoma has the opposite immunoreactivity, being negative for PAX8 and positive for CD68 (PG-M1) [68]. The gene discussed is PAX8; the disease is hereditary clear cell renal cell carcinoma.