Melanoma in xeroderma pigmentosum: 12 cases

Background. Xeroderma pigmentosum is a rare genodermatosis, with a defect a ffecting recovery of ultraviolet-induced damages and characterized by a hig h rate of malignancies malignancies of the exposed skin areas. We studied m elanoma features of patients with xeroderma pigmentosum. Patients and methods. A retrospective study of xeroderma pigmentosum patien ts admitted to the Charles Nicolle Hospital of Tunis between 1973 and 1998 Results. Two hundred sixteen patients with xeroderma pigmentosum were regis tered. Melanoma was present in 12 patients, 7 females and 5 males. Two pati ents were sisters. Cutaneous melanoma was found in 8 patients. Four patient s presented with metastatic melanoma. The median age for development of the first melanoma was 17.5 years. All of the cutaneous melanomas were found o n the face. Lentigo malignant melanoma was reported in 3 cases. The tumors were treated with surgical excision. Except for a melanoma affecting the or bit, characterized by a fatal outcome, no metastases were detected at the d ifferent investigations. Discussion. Melanoma occurs frequently in patients with xeroderma pigmentos um, it has been reported in 5.5 p. 100 of cases and 11.3 p 100 of patients with cutaneous carcinoma. The age of onset was low: 17.5 years. It appeared later than the carcinoma; The location of cutaneous melanoma in face in xe roderma pigmentosum patients indicates that they were caused mainly by sunl ight exposure. Lentigo malignant melanoma was the most frequent type. Progn osis is difficult to define owl ng to the large number of other cutaneous m alignancies. Apart from one case of rapidly fatal orbital melanoma, we reco rded long survivals even in cases of melanoma revealed by metastases.