D. Ludolph-hauser et al., Mast cells in an angiosarcoma complicating Xeroderma pigmentosum in a 13-year-old girl, J AM ACAD D, 43(5), 2000, pp. 900-902
A cutaneous angiosarcoma, a rare tumor that occurs almost exclusively in su
n-exposed skin of individuals older than 50 years, developed in an adolesce
nt with Xeroderma pigmentosum (XP). As this is the second report of a child
with angiosarcoma and XP, ultraviolet-induced DNA damage may be involved i
n the pathogenesis of this tumor Strongly increased numbers of mast cells w
ere found, particularly in the peripheral tumor area, which may reflect wit
h the requirement of mast cells for the growth of vascular structures or a
role for mast cells in the antitumor immune response.