FAMILIAL UVEAL MELANOMA

Background Familial uveal melanoma are rare and a few number of cases has been described in literature until now. An autosomal dominant inhe ritance was proposed. Material and Method From the 2169 patients exami ned and/or treated in the Jules Gonin Hospital for an uveal melanoma f rom 1969, we identified 11 patients from 9 families in which at least two members were affected. We compared the clinical data of the famili al melanoma with those of the sporadic cases of our collective with th e Kruskal-Wallis Tests. The following parameters were studied: age and sex of patients, largest and smallest tumor diameter, height and volu me of tumor, distance between tumor and optic nerve and macula, distan ce of tumor to the equator. Age and sex were also compared with litera ture. Results Among the 11 patients, nine were treated with an acceler ated proton beam, one with Cobalt(60) applicator and one has been enuc leated. The median age was 58 years among the probands and 54.6 years among the sporadic cases of the collective. Sex ratio is superposable between both groups: male 45.5%, female 54.5% among the probands and m ale 48.6%, female 51.4% among the sporadic cases. The comparison betwe en both groups with the Kruskal-Wallis Tests did not show any signific ant statistical difference. Conclusion An uveal melanoma occurring at least in two members of the same family is not due to chance alone? Ge netic factors associated to external factors are probably responsable.