A Case of Childhood Dermatofibrosarcoma Protuberans / 대한피부과학회지

ABSTRACT

Dermatofibrosarcoma protuberans(DFSP) is a rare cutaneous tumor, which occurs most commonly on the trunk or the proximal extremities, affects all races and often develops between the second and fifth decades of life. It is uncommon in childhood and is sometimes mistaken for a vascular lesion, as it often presents as a blue macule or small nodule. Histologically, it shows large uniformed spindle shaped cells arranged in a cartwheel or storiform pattern. Recently, the immunohistochemical staining with monoclonal antibody to CD34 is reported to give assistance in the differential diagnosis of DFSP from the fibrous or neural tumors. We report herein a case of childhood DFSP showing CD34 positivity in a 9-year-old girl