In contrast to these somatic mutations, germline mutations affecting highly conserved amino acids have been reported for the fumarate hydratase-encoding gene (FH) on chromosome 1q43 in tumor syndromes (Alam et al., 2001; Tomlinson et al., 2002) but only in rare cases of non-syndromic UL (Barker et al., 2002; Kiuru et al., 2002). The gene discussed is FH; the disease is neoplastic syndrome.