It has been reported that more than 130 different germline mutations, including missense, frameshift, indel, splice site, and nonsense mutations, are involved in HLRCC patients.[6] For example, missense mutations in the FH could lead to the expression of nonfunctional proteins that are findable by immunohistochemical staining, leading to false positive results in FH.[15] As a result, molecular genetic testing is still the gold standard to confirm HLRCC. This evidence concerns the gene FH and hereditary leiomyomatosis and renal cell cancer.