Cutaneous angiosarcoma in a patient with xeroderma pigmentosum.
Xeroderma pigmentosum (XP) is a rare, autosomal recessive disorder characterized by photosensitivity, cutaneous pigmentary changes, premature skin ageing and development of various cutaneous and internal malignancies at an early age as a result of a defect in nucleotide excision repair following ultraviolet light exposure. Cutaneous angiosarcomas are aggressive neoplasms that are rarely associated with XP. In this communication, we report the case of a 40-year-old male patient with XP who developed an angiosarcoma of the face and discuss the implications of this association in view of recent developments in this field.
Arora
Raman
R
Department of Pathology, All India Institute of Medical Sciences, New Delhi 110 029, India.
Sharma
Alok
A
Gupta
Ruchika
R
Vijayaraghavan
M
M
D000328:Adult; D006394:Hemangiosarcoma / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D012878:Skin Neoplasms / Q000150:complications* / Q000473:pathology; D014983:Xeroderma Pigmentosum / Q000150:complications* / Q000473:pathology