BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade fibrohistiocytic tumor that usually occurs on the trunk or proximal extremities and typically appears during the second to fifth decade of life. It most commonly begins as a red-blue plaque that grows slowly and ultimately becomes nodular. The tumor is associated with a high recurrence rate but low metastatic potential. ...

The genetic changes leading to the development of malignant peripheral nerve sheath tumors (MPNSTs) are largely unknown. The few tumors that have been investigated cytogenetically had highly complex karyotypes and no consistent rearrangements, and the attempts to pinpoint consistent DNA-level changes have met with only limited success. We used comparative genomic hybridization to analyze seven ...

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with tendency to recur locally and only rarely metastasizes to vital organs. Surgery with wide margins remains the standard treatment. DFSP is characterized by specific chromosomal abnormalities involving the platelet derived growth factor B locus (PDGFB). In the majority of cases a supernumerary ring chromosome containing amplified ...

68 Correspondence To the Editor: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous soft tissue neoplasm of intermediate-to-low grade malignant potential. It arises from the dermis and invades locally into deeper subcutaneous tissue. It is characterized by a high local recurrence rate after standard surgical excision, partly due to asymmetric infiltration of the subcutis by delicate strands ...

267 simultaneous occurrence with a malignant giant cell tumor of soft parts. Cancer 1993;72:462-8. 3. Morrison AE, Lang PG. Case of rapidly enlarging Dermatofibrosarcoma protuberans during pregnancy followed by metastasis in the absence of local recurrence. Dermatol Surg 2006;32:125-7. 4. Angouridakis N, Kafas P, Jerjes W, et al. Dermatofibrosarcoma protuberans with fibrosarcomatous transformat...

COL1A1: collagen 1 a1 DF: dermatofibroma DFSP: dermatofibrosarcoma protuberans FISH: fluorescence in situ hybridization JXG: juvenile xanthogranuloma PDGFB: platelet-derived growth factor-b INTRODUCTION Spindle cell tumors occur in the pediatric population and can prove to be aggressive clinically, as in the case with dermatofibrosarcoma protuberans (DFSP). Soft tissue sarcomas represent less t...

INTRODUCTION Excision of large dermatofibrosarcoma protuberans in the anterior aspect of the trunk often results in large surgical defects that frequently dictate the need for microsurgical reconstruction. However, this option is not always available. PRESENTATION OF CASE The authors describe two patients with very large anterior trunk dermatofibrosarcoma protuberans: one in the epigastric re...