Editor,—Uveal melanoma is the most common primary malignant intraocular tumour in adults, representing 70% of all malignant ocular tumours.1

They appear sporadically in the absence of clear predisposing genetic factors. However, the family history of some patients suggests that there could be a genetic basis.2 Some cases of family uveal melanoma have been described in the literature, and they point to a dominant autosomal hereditary transmission.34

The family uveal melanoma accounts for only 0.6% of patients with uveal melanoma. Considering the low incidence of uveal melanoma in the general population, the possibility of developing uveal melanoma in a family context is very low. Since the first description by Silcock in 1892 of the case of a mother and her two daughters affected with this illness, only 51 families had been reported until 1996.45

Unidentified mutations on the germinal line might be involved in its pathogenesis.46 There are several reports of simultaneous occurrence of uveal melanoma and breast cancer. Some of them are related to one of the genes already known as predisposing to breast and ovary cancer, the “BRCA2.” 78

Even though there is no demonstration of an implicated gene, many studies suggest that the occurrence of family uveal melanoma is not just a coincidence.9

Three clinical cases of histopathologically proved intraocular malignant melanoma involving first generation members of the same family (siblings) are analysed, and their evolution is reported.