Outcome of wide local excision in dermatofibrosarcoma protuberans and use of radiotherapy for margin-positive disease

Abstract

Purpose:Wide local excision (WLE) is the preferred treatment of dermatofibrosarcoma protuberans (DFSP). The aim is to achieve negtive margins. We followed the impact of radiotherapy used postoperatively for both margin-negative and margin-positive DFSP tumors.Materials and Methods:Outcome of treatment of 36 patients of DFSP treated at our hospital was assessed. Thirty patients received radiotherapy postoperatively and six patients received radiotherapy alone. The maximum dimension of the lesion was 15 cm2. Patients were followed up for varying periods of time for any recurrence.Results:10-year actuarial local control rate was determined. Local control was realized in six patients who were treated with radiotherapy alone. 30 patients were treated by radiotherapy and surgery. Out of these 30 patients, there were 6 local failures (failure rate 10%). Actuarial control rate was 82%. The failures were among patients who had positive margins.Conclusion:Radiotherapy is effective, and it decreases the recurrence rate in the treatment of DFSP. It is especially helpful in margin-positive disease. This appears true for patients treated with radiotherapy alone or radiotherapy used postoperatively.

The Role of Postoperative Radiotherapy in the Management of Dermatofibrosarcoma Protuberans: A Multidisciplinary Systematic Review

“…Hamid et al, 2013 [ 35 ] described thirty-six patients diagnosed with DFSP (twenty-six male patients and ten female patients with a mean age of 38 years), of whom thirty patients underwent surgical treatment and postoperative adjuvant radiotherapy, and six patients were treated with radiotherapy alone. The head and neck were involved in three cases, the trunk was affected in twenty cases, twelve patients had the lesion in the extremities and, in one case, the external genitalia were involved.…”

Management of dermatofibrosarcoma protuberans

“…MD Anderson demonstrated patients with a 98% disease free survival at 5 years and 93% at 10 years, respectively [39]. Adjuvant RT has been shown to be especially effective in patients with positive margins, but administration is still patient specific [40].…”

Large anterior abdominal wall fibrosarcoma in a known case of recurrent dermatofibrosarcoma protuberans

“…6,15 Adjuvant radiotherapy (RT), administered either before or after surgery, significantly reduces the risk of local recurrence in patients who have positive margins or unsatisfactory surgery. 16,17 Radiotherapy may also be used in cases of recurrence, metastasis and select inoperable cases. Maki et al, first showed that cells transformed with the collagen type 1 alpha 1-platelet derived growth factor beta chain (COL1A1-PDGFB) are inhibited by the tyrosine kinase inhibitor imatinib mesylate.…”