Interestingly, familial uveal melanoma is associated with germline mutations of other DNA repair genes, such as PALB2 (partner and localizer of BRCA2; involved in breast cancer), which leads to homologous recombination defects, or MLH1 (mutL homolog 1; part of Lynch syndrome), which leads to microsatellite instability (MSI) [16]. Here, MLH1 is linked to Lynch syndrome.