Loss of PAX8 phenocopied expression patterns in renal TSC-related PEComas (angiomyolipomas), which show striking downregulation of PAX8 or PAX2 and their core target genes (GATA3, WT1, LHX1)36,52 compared to surrounding kidney parenchyma. This evidence concerns the gene PAX8 and neoplasm with perivascular epithelioid cell differentiation.