MALIGNANT-MELANOMA IN XERODERMA-PIGMENTOSUM PATIENTS - REPORT OF 5 CASES

Xeroderma pigmentosum is a rare genetic disease transmitted via a rece ssive gene with an altered reaction of the epidermis to light. Fifty p er cent of patients develop a skin tumour by 8 years of age. The major ity of patients mag have multiple tumours, but metastasis is rare. In the last 25 years me have treated 24 xeroderma pigmentosum patients in our clinic. Only five patients had developed cutaneous malignant mela noma during their follow-up. Three of the patients mere from the same family, melanoma occurring in three of five affected individuals. All xeroderma pigmentosum patients with malignant melanoma had received cl assical treatment modalities. Except one case of fulminant pattern, al l four patients had long disease-free survival. Although early detecti on and treatment of these cutaneous malignancies will reduce morbidity and mortality, genetic counselling remains the most important protect ive measure for xeroderma pigmentosum.