Stivala Alessio, Lombardo Giuseppe A G, Pompili Gianluca, Tarico Maria Stella, Fraggetta Filippo, Perrotta Rosario Emanuele
Department of Medical and Surgery Specialties, Section of Plastic Surgery;
Oncol Lett. 2012 Nov;4(5):1047-1055. doi: 10.3892/ol.2012.887. Epub 2012 Aug 30.
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.
隆突性皮肤纤维肉瘤(DFSP)是一种罕见的具有中度恶性的软组织肿瘤。它最初位于皮肤,可由此浸润深部结构,并且在切除不充分时易局部复发。t(17;22)(q22;q13)染色体易位是导致DFSP发病的主要细胞遗传学改变。治疗选择包括通过进行切缘宽度大于3 cm的传统手术或莫氏显微外科手术来完整切除肿瘤。我们整形与重建外科进行了一项回顾性研究,所有数据均收集自该科室1999年至2011年期间59例DFSP患者的病历。59例中有13例(22%)采用传统切除术治疗;3例(5%)切缘无肿瘤,8例(14%)需要手术修正,2例(3%)导致复发。59例中有46例(78%)采用广泛切除术治疗;43例(73%)切缘无肿瘤,3例(5%)需要手术修正,0例(0%)导致复发。总之,所收集的数据揭示了围绕采用安全切缘一般指南的争议。需要进一步研究来调查从可能外观上健康的切缘获得基因型改变切缘的可能性。