Atypical fibroxanthoma and squamous cell carcinoma of the conjunctiva in xeroderma pigmentosum.
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.
Shao
Lei
L
Department of Pathology, Children's Mercy Hospitals and Clinics, 2401 Gillham Road, Kansas City, MO 64108, USA. lshao@cmh.edu
Newell
Brandon
B
Quintanilla
Norma
N
D001741:Black or African American; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D002294:Carcinoma, Squamous Cell / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D002675:Child, Preschool; D003228:Conjunctiva / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D005500:Follow-Up Studies; D018219:Histiocytoma, Benign Fibrous / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome; D016159:Tumor Suppressor Protein p53 / Q000378:metabolism; D014746:Vimentin / Q000378:metabolism; D014983:Xeroderma Pigmentosum / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D000097382:CD68 Molecule
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D016159:Tumor Suppressor Protein p53; D014746:Vimentin; C067980:CD68 antigen, human; D000097382:CD68 Molecule