FH-dRCC is the preferred term for RCC with compatible morphology, negative FH immunohistochemical (IHC) staining, positive 2-SC IHC staining, and/or identification of a mutation in the FH gene in the tumor, when the clinical and family history of CL and MUL is uncertain and the genetic status is unknown [8]. The gene discussed is FH; the disease is neoplasm.