Dermatofibrosarcoma protuberans: Our experience of 59 cases

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 trans-locatio...

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Bibliographic Details
Published in:Oncology letters 2012-11, Vol.4 (5), p.1047-1055
Main Authors: STIVALA, ALESSIO, LOMBARDO, GIUSEPPE A.G, POMPILI, GIANLUCA, TARICO, MARIA STELLA, FRAGGETTA, FILIPPO, PERROTTA, ROSARIO EMANUELE
Format: Article
Language:English
Subjects:
conventional excision
dermatofibrosarcoma protuberans
imatinib
Kinases
Medical records
Metastasis
Mohs surgery
Oncology
Patients
Scars
Signal transduction
Studies
Surgery
wide excision
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Summary: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 trans-location 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.
ISSN:1792-1074
1792-1082
DOI:10.3892/ol.2012.887