Dermatofibrosarcoma protuberans: Our experience of 59 cases

Abstract

Abstract. Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor with intermediate malignancy. It is initially located on the skin from where it is able to infiltrate the deep structures and has a tendency to recur locally following inadequate excision. A t(17;22)(q22;q13) chromosome translocation is the main cytogenetic alteration responsible for the onset of DFSP. Treatment options include complete surgical excision by performing conventional surgery with wide margins (>3 cm) or Mohs micrographic surgery. A retrospective study was conducted in our Department of Plastic and Reconstructive Surgery and all data were collected from medical records of 59 DFSP patients within this department from 1999 to 2011. A total of 13 of 59 (22%) cases were treated with conventional excision; 3 (5%) cases resulted in tumor-free margins, 8 (14%) cases required surgical revision and 2 (3%) cases lead to recurrence. A total of 46 of 59 (78%) cases were treated with wide excision; 43 (73%) cases resulted in tumor-free margins, 3 (5%) cases required surgical revision and 0 (0%) cases lead to recurrence. In conclusion, the data collected reveal the controversy surrounding the adoption of general guidelines regarding safe margins. Further studies are required to investigate the possibility of obtaining genotypically altered margins from margins that may appear phenotypically healthy.

Clinicopathological features of dermatofibrosarcoma protuberans

“…Certain studies have advocated the use of Mohs micrographic surgery with incremental excision until normal tissue is obtained, as documented by repeat frozen sections (13). Several studies have demonstrated a significant correlation between a wide excision and a low recurrence rate (9,13). In the present study, the recurrence of DFSP occurred in 26 cases (38.2%).…”

“…However, CD34 immunohistochemical positivity is also expressed by other spindle cell tumors, including solitary fibrous tumors, sclerotic fibroma, superficial acral fibromyxoma, cellular digital fibroma, dermatofibroma and nuchal-type fibroma. Additional immunohistochemical staining of factor XIIIa, stromelysin III, CD44, CD163 and D2-40 have been found to be positive in dermatofibroma and negative in DFSP (1)(2)(3)(4)(6)(7)(8)(9)(10). Spindle tumor cells are immunonegative for S100 protein, smooth muscle actin, desmin, cytokeratin and epithelial membrane antigen (1)(2)(3)(4).…”

Wide local excision vs. Mohs Tübingen technique in the treatment of dermatofibrosarcoma protuberans: a two‐centre retrospective study and literature review

“…1,[9][10][11] Median age at diagnosis is 20-59 years 1 ; however, congenital and paediatric cases have also been described. 2,[12][13][14][15][16] As for all soft tissue sarcomas, DFSP has no specific risk factors; it arises generally on healthy skin but occasionally can develop on chronically damaged areas. 5,16 Despite its rapid progression during pregnancy, it is not demonstrated an eventually hormonal origin.…”

“…2,[12][13][14][15][16] As for all soft tissue sarcomas, DFSP has no specific risk factors; it arises generally on healthy skin but occasionally can develop on chronically damaged areas. 5,16 Despite its rapid progression during pregnancy, it is not demonstrated an eventually hormonal origin. 17 The malignant potential of DFSP is considered as intermediate between that of dermatofibroma and malignant fibrous histiocytoma.…”

Tratamiento quirúrgico de los tumores de la pared abdominal

“…Dentro de las técnicas quirúrgicas disponibles están la exéresis simple, la exéresis amplia y la cirugía micrográfica de Mohs, aunque existe cierta controversia entre estas 2 últimas. En la actualidad, independientemente de la téc-nica que se elija, se recomiendan unos márgenes libres de resección de 2-3 cm más allá de los límites macroscópicos visibles del tumor, a veces incluyendo la fascia muscular subyacente [13][14][15] . A pesar de las escisiones locales amplias, todavía se observan tasas de recurrencia local próximas al 20 % 10 .…”