Dermatofibrosarcoma protuberans: pathology, genetics, and potential therapeutic strategies

Dermatofibrosarcoma protuberans (DFSP), the most common dermal sarcoma, is a malignant fibroblastic tumor most frequently arising in middle-aged adults. It is typically a low-grade sarcoma that grows slowly but has a high rate of local recurrence with low metastatic potential. Dermatofibrosarcoma protuberans is characterized by a specific translocation t(17;22)(q22;q13) leading to the formation of COL1A1-PDGFB fusion transcripts. Histologically, DFSP has characteristic morphology, of storiform islands of bland spindle cells, and immunohistochemically, it shows diffuse expression of CD34.

Source: Annals of Diagnostic Pathology - September 25, 2016 Category: Pathology Authors: Khin Thway, Jonathan Noujaim, Robin L Jones, Cyril Fisher Source Type: research