Additionally, six patients had FH P/LP variants (p.Gln376Pro (n = 3), p.His402Tyr (n = 2), p.Gly397Arg (n = 1)) that have been reported in homozygous and compound heterozygous patients with fumarate hydratase deficiency, but have not, to our knowledge, been reported in patients with HLRCC. Here, FH is linked to hereditary leiomyomatosis and renal cell cancer.