ABSTRACT
D ermatofibrosarcoma Protuberans (DP), also named Darier-Ferrand tumor, is a soft tissue tumor typi cally located in the deep dermis. It arises at any site but preferentially in the subcutane ous tissue of the trunk and proximal extremi ties. DP occurs during early and mid-adult life.1 Although authentic DP may also but seldomly, occurs in children, a distinct entity, giant cell fibroblastoma (GCF), has been con sidered as a juvenile form of D P 1 2 DP is locally invasive and can be classed among tumors o f low or borderline malignancy. Current therapy is purely surgical. The overall high recurrence rate, up to 50% and the repeti tion o f recurrences are common, reflecting the extensive infitrative growth o f the tumor and the difficulty to recognize it at the time of the surgery.1,3 To avoid recurrence, such wide mar gins are required that secondary reconstmctive surgery and skin grafts are often needed. An accurate diagnosis differentiating DP from other tumors, benign or malignant, is there fore crucial. It is sometimes particularly puz zling, requiring highly reliable diagnostic markers as are recurrent chromosome trans locations and their subsequent specific molecu lar rearrangements.4,5 In this context, we inves tigated the features of D P as part o f a program on cytogenetic and molecular characterization o f soft tissue tumors.
