Nevoid basal cell carcinoma syndrome: combined etretinate and surgical treatment.
Aromatic retinoids have been shown to be useful in the management of various genodermatoses, including nevoid basal cell carcinoma syndrome (NBCCS). A patient with this condition was treated by combined etretinate and surgical therapy. Over the course of 12 months, the patient was placed on oral etretinate therapy, with a dosage of 1 mg/kg/day for 3 months, and 0.5 mg/kg/day for 9 months. In the first period, notable tumor reduction was observed in the larger tumors. Complete clinical regression was seen in only 26% of BCCs with an overall tumor size of less than 1 cm in diameter. In the second period, no tumor changes were observed, but no new tumors appeared. Surgical excisions of the remaining tumors were carried out 3 months after the end of the etretinate treatment, with excellent results and no recurrences after 6 months of follow-up. Treatment with etretinate permits a less aggressive surgical excision and recurrence rate than surgical excision alone.
Sanchez-Conejo-Mir
J
J
Department of Medical Surgical Dermatology and Venereology, University Hospital, Seville, Spain.
Camacho
F
F
D001478:Basal Cell Nevus Syndrome / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D002280:Basal Cell Carcinoma / Q000628:therapy*; D003131:Combined Modality Therapy; D005050:Etretinate / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008875:Middle Aged; D012878:Skin Neoplasms / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*
D005050:Etretinate