Cutaneous angiosarcoma in a patient with xeroderma pigmentosum

Pediatr Hematol Oncol. 2004 Jan-Feb;21(1):23-6.

Abstract

Xeroderma pigmentosum (XP) is a DNA repair defect syndrome associated with an increased risk to developing skin neoplasms on sun-exposed cutaneous surfaces. This report describes the case of a 15-year-old boy with XP who developed cutaneous angiosarcoma. The patient was cured with surgery alone, despite incomplete resection, and he is alive without evidence of disease 40 months after diagnosis. It is the fourth reported case--and the third in pediatric age--of the association of XP with this soft part sarcoma.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • DNA Repair / radiation effects
  • Hemangiosarcoma / complications*
  • Hemangiosarcoma / pathology
  • Hemangiosarcoma / surgery
  • Humans
  • Male
  • Skin Neoplasms / complications*
  • Skin Neoplasms / pathology
  • Skin Neoplasms / surgery
  • Sunlight / adverse effects
  • Xeroderma Pigmentosum / complications*
  • Xeroderma Pigmentosum / pathology