Atypical fibroxanthoma presenting in a patient with stage III mycosis fungoides.

The association of atypical fibroxanthoma (AFX) and mycosis fungoides (MF) has never been reported. We report a 77-year-old female who was referred for Mohs micrographic surgery (MMS) of an AFX on the right hand. At the time of presentation, this patient had stage III MF, with erythroderma and palpable lymph nodes. Our patient's AFX measured 1.5x2.8 cm. The tumor was removed by MMS with clear margins. The pathologic specimen revealed a relatively well-circumscribed neoplasm in the upper dermis composed of atypical spindle cells with pleomorphism, hyperchromatic nuclei, and brisk mitotic activity. Immunohistochemical stains were focally positive for CD68, and negative for S-100, Melan-A, desmin, smooth muscle actin and neurofilament. Perilesional skin revealed epidermal hyperplasia, parakeratosis, and multiple epidermotropic lymphocytes with enlarged and hyperchromatic nuclei. One year after the initial MMS, our patient developed another tumor on the right hand, which measured 4x6 mm. This neoplasm underwent a fusiform excision with clear margins. Histological examination revealed evidence of MF and an AFX. To our knowledge, this is the first report of an AFX arising in a patient with MF.