Atypical fibroxanthoma and squamous cell carcinoma of the conjunctiva in xeroderma pigmentosum

Pediatr Dev Pathol. Mar-Apr 2007;10(2):149-52. doi: 10.2350/06-06-0103.1.

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.

Publication types

  • Case Reports

MeSH terms

  • African Americans
  • Antigens, CD / metabolism
  • Antigens, Differentiation, Myelomonocytic / metabolism
  • Carcinoma, Squamous Cell / metabolism
  • Carcinoma, Squamous Cell / pathology*
  • Carcinoma, Squamous Cell / surgery
  • Child, Preschool
  • Conjunctiva / metabolism
  • Conjunctiva / pathology*
  • Conjunctiva / surgery
  • Follow-Up Studies
  • Histiocytoma, Benign Fibrous / metabolism
  • Histiocytoma, Benign Fibrous / pathology*
  • Histiocytoma, Benign Fibrous / surgery
  • Humans
  • Immunohistochemistry
  • Male
  • Skin Neoplasms / metabolism
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / surgery
  • Treatment Outcome
  • Tumor Suppressor Protein p53 / metabolism
  • Vimentin / metabolism
  • Xeroderma Pigmentosum / metabolism
  • Xeroderma Pigmentosum / pathology*
  • Xeroderma Pigmentosum / surgery

Substances

  • Antigens, CD
  • Antigens, Differentiation, Myelomonocytic
  • CD68 antigen, human
  • Tumor Suppressor Protein p53
  • Vimentin