Cutaneous angiosarcoma in a patient with xeroderma pigmentosum.
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.
Marcon
Ilaria
I
Pediatric Oncology Unit, Istituto Nazionale Tumori, Milan, Italy.
Collini
Paola
P
Casanova
Michela
M
Meazza
Cristina
C
Ferrari
Andrea
A
D000293:Adolescent; D004260:DNA Repair / Q000528:radiation effects; D006394:Hemangiosarcoma / Q000150:complications* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D012878:Skin Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D013472:Sunlight / Q000009:adverse effects; D014983:Xeroderma Pigmentosum / Q000150:complications* / Q000473:pathology