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CUTANEOUS ANGIOSARCOMA IN A PATIENT WITH XERODERMA PIGMENTOSUM 

Authors: Ilaria Marcon a;  Paola Collini b;  Michela Casanova a;  Cristina Meazza a; Andrea Ferrari a
Affiliations:   a Pediatric Oncology Unit, Istituto Nazionale Tumori, Milan, Italy
b Pathology Department, Istituto Nazionale Tumori, Milan, Italy
DOI: 10.1080/08880010490263380
Publication Frequency: 8 issues per year
Published in: journal Pediatric Hematology and Oncology, Volume 21, Issue 1 January 2004 , pages 23 - 26
Number of References: 7
Formats available: HTML (English) : PDF (English)
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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.
Keywords: cutaneous angiosarcoma; xeroderma pigmentosum
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