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  • Title: Atypical fibroxanthoma and squamous cell carcinoma of the conjunctiva in xeroderma pigmentosum.
    Author: Shao L, Newell B, Quintanilla N.
    Journal: Pediatr Dev Pathol; 2007; 10(2):149-52. PubMed ID: 17378688.
    Abstract:
    Patients with xeroderma pigmentosum (XP) have defective DNA repair and a high predisposition to developing abnormalities and neoplasia in the sun-exposed areas of the skin and mucous membranes. The most common tumors reported in patients with XP are squamous cell carcinomas, basal cell carcinomas, and melanomas. Atypical fibroxanthoma (AFX) is a pleomorphic tumor that arises predominantly in the sun-damaged skin of the head and neck regions of the elderly. We describe a unique case of a 6-year-old African American boy with XP who developed an atypical fibroxanthoma and 2 squamous cell carcinomas in the conjunctiva. The clinical and histopathologic findings of AFX are discussed.
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