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.
Cutaneous angiosarcoma in a patient with xeroderma pigmentosum / I. Marcon, P. Collini, M. Casanova, C. Meazza, A. Ferrari. - In: PEDIATRIC HEMATOLOGY AND ONCOLOGY. - ISSN 0888-0018. - 21:1(2004), pp. 23-26.
Cutaneous angiosarcoma in a patient with xeroderma pigmentosum.
A. Ferrari
2004
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.
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