CASE REPORT

Congenital dermatofibrosarcoma protuberans with fibrosarcomatous and myxoid change

W Gu^1,3, A Ogose¹, H Kawashima¹, H Umezu², N Kudo¹, T Hotta¹ and N Endo¹

¹ Division of Orthopaedic Surgery, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-Dori, Niigata, 951-8510 Japan
² Division of Pathology, Niigata University Hospital, Niigata, Japan
³ Division of Orthopaedic Surgery, First Affiliated Hospital of Harbin Medical University, Harbin, China

Correspondence to:
Correspondence to:
Dr A Ogose
Division of Orthopaedic Surgery, Niigata University Graduate School of Medical and Dental Sciences, 1-757 Asahimachi-Dori, Niigata, 951-8510 Japan; aogose{at}med.niigata-u.ac.jp

This report describes a case of congenital dermatofibrosarcoma protuberans (DFSP) with fibrosarcomatous (FS) and myxoid areas. Immunohistochemical results showed that tumour cells in ordinary DFSP areas were diffusely positive for CD34, whereas in the FS and myxoid areas, few tumour cells were positive for this antigen. Ki-67 positive tumour cell numbers were greater in the FS (11.8%) and myxoid areas (19.8%) relative to ordinary DFSP areas (2.2%). Reverse transcription polymerase chain reaction and sequence analysis showed the presence of an identical COL1A1–PDGFB fusion transcript in ordinary DFSP (plaque-like area), FS, and myxoid areas of DFSP. These results indicate that the three components of DFSP have a common histogenesis. This study documents the first application of gene analysis involving the myxoid area of DFSP.

Abbreviations: DFSP, epithelial membrane antigen; DFSP-FS, dermatofibrosarcoma protuberans with fibrosarcomatous area; FS, fibrosarcomatous

Keywords: dermatofibrosarcoma; fibrosarcoma; myxoid; gene fusion; cytogenetic analysis

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• Szollosi, Z., Scholtz, B., Egervari, K., Nemes, Z. (2007). Transformed dermatofibrosarcoma protuberans: real time polymerase chain reaction detection of COL1A1-PDGFB fusion transcripts in sarcomatous areas. J. Clin. Pathol. 60: 190-194 [Abstract] [Full Text]