Atypical fibroxanthoma arising in a young patient with Li-Fraumeni syndrome.

摘要: Patients with Li-Fraumeni syndrome (LFS) have a germ-line mutation of p53 (TP53) and are predisposed to develop variety malignancies at an early age. In this report, we describe 18-year-old woman LFS who developed atypical fibroxanthoma (AFX) on her left arm. This tumor was based in the dermis, sparsely cellular had ill-defined borders. It composed predominantly medium-sized spindled-shaped cells, but many large cells pleomorphic nuclei were also present. Immunohistochemical stains showed that lacked expression keratin, S-100 protein, desmin CD34. Array-based comparative genomic hybridization (aCGH) revealed marked instability multiple whole chromosome losses, including chromosomes 8, 10, 13 22, as well partial loss 17p. represents one few reports cutaneous patient rare example AFX occurring young