Cutaneous angiosarcoma in an adolescent girl with Xeroderma Pigmentosum: A case report
DOI:
https://doi.org/10.35509/01239015.720Keywords:
Xeroderma Pigmentoso. Angiosarcoma. Children. Radiation therapy, ChemotherapyAbstract
Xeroderma Pigmentosum (XP) is a rare, autosomal recessive disorder characterized by extreme sensitivity to ultraviolet radiation (UVR) from sunlight that produces a defective repair of DNA damage and, as a consequence, a marked predisposition to the development of cancer of the skin. Clinically it is manifested by photosensitivity, pigmentary skin changes, premature aging of the skin. This disorder affects approximately 1 in 250,000 inhabitants per year in the United States. We present the case of a 12-year-old patient with a Xeroderma Pigmentosum detected at 6 months who developed an ulcerated lesion in the left nasal slope with a report of Angiosarcoma (AS) pathology with positive CD31, EGR and CD34; who was treated with wide resection of the lesion and adjuvant chemotherapy with Plaquitaxel and Doxorubicin without radiotherapy.
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