[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 inheritance was proposed.

MATERIAL AND METHOD

From the 2169 patients examined and/or treated in the Jules Gonin Hospital for an uveal melanoma from 1969, we identified 11 patients from 9 families in which at least two members were affected. We compared the clinical data of the familial melanoma with those of the sporadic cases of our collective with the Kruskal-Wallis Tests. The following parameters were studied: age and sex of patients, largest and smallest tumor diameter, height and volume of tumor, distance between tumor and optic nerve and macula, distance of tumor to the equator. Age and sex were also compared with literature.

RESULTS

Among the 11 patients, nine were treated with an accelerated proton beam, one with Cobalt60 applicator and one has been enucleated. 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 male 48.6%, female 51.4% among the sporadic cases. The comparison between both groups with the Kruskal-Wallis Tests did not show any significant statistical difference.

CONCLUSION

An uveal melanoma occurring at least in two members of the same family is not due to chance alone. Genetic factors associated to external factors are probably responsable.