Abstract

PRADA, Camila et al. Cutaneous angiosarcoma in an adolescent girl with Xeroderma Pigmentosum: A case report. rev.colomb.cancerol. [online]. 2022, vol.26, n.1, pp.111-116.  Epub Sep 03, 2022. ISSN 0123-9015.  https://doi.org/10.35509/01239015.720.

Xeroderma Pigmentosum is a rare autosomal recessive disorder characterized by extreme sensitivity to ultraviolet radiation (UVR) from sunlight that results in a defective repair of DNA damage and, as a consequence, a marked predisposition to the development of cancer of the skin. Its clinical manifestations are photosensitivity, pigmentary skin changes, and premature aging of the skin. This disorder affects approximately 1 in 250,000 individuals per year in the United States. We present the case of a 12-year-old patient with Xeroderma Pigmentosum detected at 6 months of age, who developed an ulcerated lesion on the left nasal slope with a pathology report of angiosarcoma (AS), which we positive for ERG, CD31, and CD34. The patient was treated with wide resection of the lesion and adjuvant chemotherapy with paclitaxel and doxorubicin without radiation therapy.

Keywords : Xeroderma Pigmentosum; angiosarcoma; children; radiation therapy; chemotherapy.

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