[Topical and systemic chemotherapy with 5-fluouracil in facial carcinoma secondary to xeroderma pigmentosum]

to evaluate the feasibility, tolerance/toxicity and therapeutic efficacy of 5-fluorouracil (5-FU) by topical application and systemic use, in facial carcinoma associated with XP.

This is a prospective study of 10 patients with a median age of 22.9 years and a sex ratio of 4. Tumour lesions were facial mainly in the jugal and temporal region (36%). Chemotherapy indication was discussed in multidisciplinary committee, the topical 5-fluorouracil was applied locally twice a day, whereas the systemic treatment consisted of FUFOL protocol (every 4 weeks a combinaison of a short perfusion of 340 mg/m2 5-FU and preceded by an infusion of 20mg/m2 of folic acid, day 1 to 5); or C-FU protocol, combining continuous infusion of 5-FU (1 g/m2) 5 days associated with cisplatin (100 mg/m2, day 1) every 3 weeks.

The median topical treatment duration was of 12 months in 10 patients. We noted a full tumoral regression in 10% of cases. Concerning systemic treatment, the median number of FUFOL cycles was 4 (2 to 6) and we observed a complete response in 6 patients (60%), partial in 2 cases (20%). Treatment was well tolerated in most cases except for the cutaneous irritation on 5-FU application zone and a 4 grade cisplatin otoxicity.

Systemic or topical chemotherapy represents an interesting palliative option for facial carcinoma associated with XP, avoiding reiterated surgery and its cosmetic consequences.