关键词: 着色性干皮症, 颌面部, 肿瘤，继发原发性, 外科手术, 预后

Abstract: Objective　 To analyze the clinical characteristics, surgical treatment, pathology and prognosis of maxillofacial malignancy secondary to Xeroderma Pigmentosum in MNAZI?MMOJA hospital of Zanzibar . Methods This was a retrospective case study. 8 maxillofacial malignancy patients secondary to Xeroderma Pigmentosum visiting Zanzibar MNAZI?MMOJA hospital between 2012 and 2014 were reviewed. The data of the 8 patients including the surgical treatment, pathology and prognosis were analyzed in the study. Result The result of pathological and immunohistochemical examination suggested that 4 cases had squamous cell carcinoma, 3 cases had basal cell carcinoma, and 1 case had malignant melanoma. All these 8 patients were followed-up for 6-24months. 1 tongue cancer patient died nine months later after operation; 1 patient giving up treatment died half a year later; there was no recurrence for other 6 patients. Conclusion Maxillofacial tumors secondary to Xeroderma Pigmentosum are usuallymalignant, and tend to recur. The most common two are Basal Cell Carcinoma and Squamous Cell Carcinoma. Although the characteristics of this disease include a rapidly progressive clinical course in the maxillofacial region and a poor prognosis,timely surgical treatment can significantly improve the prognosis. The range of the surgical treatment should be large enough, and skin grafting or neighbor flap can be performed after massive resection of the tumor.

Key words: Xeroderma Pigmentosum, maxillofacial region, Neoplasms, second primary, surgical procedure, Prognosis