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. The gene discussed is GATA3; the disease is neoplasm with perivascular epithelioid cell differentiation.