Although data in lung and GEP-NETs arising in the context of Von-Hippel Lindau syndrome (VHL) are lacking, there is evidence that mutations of multiple chromatin remodelers including the histone methyltransferase SETD2 and the histone demethylases UTX and JARID1C may contribute to the progression of VHL-associated clear renal cell carcinoma [23]. Here, VHL is linked to von Hippel-Lindau disease.