Management of dermatofibrosarcoma protuberans

Abstract

Purpose of review Provide an up to date review of the diagnosis, workup and treatment of dermatofibrosarcoma protuberans (DFSP). DFSP can be a challenging disease to manage and adequate understanding of the most up to date literature can help provide comprehensive treatment strategies. Recent findings DFSP is an infiltrative cutaneous sarcoma. It tends to have deep local invasion with a high risk of local recurrence, but a low risk of distant metastasis. It presents typically as a slow growing, asymptomatic skin lesion. It presents rarely in the head and neck, only 15% of the time. Recent data has discussed the role of wide local excision (WLE) vs. Mohs surgery. In addition, for unresectable disease the role of systemic therapy and immunomodulatory agents such as Imatinib has shown success. Summary Typically, surgical management is the first line for DFSP, however the risk for local recurrence still remains high with negative margins. Due to this risk, lifelong surveillance is required after initial diagnosis and management. Similar to other head and neck tumors, most recurrences happen within the first 3 years after treatment. DFSP can be treated with WLE or Mohs. For aggressive disease that is considered unresectable systemic therapy does exist, including molecular targeted therapies.

Atrophic dermatofibrosarcoma protuberans: Two case reports and literature review

“…Mohs and slow Mohs) as viable options ( 10 , 11 ). For cases with recurrent, unresectable, and metastatic tumors, radiation therapy and targeted immunotherapy such as tyrosine kinase inhibitors have been shown to be effective ( 12 ). Imatinib mesylate can competitively inhibit ATP binding to the PDGF-β receptor, which slows down kinase activity, limits the growth of the tumor, and promotes apoptosis ( 13 ).…”

Dermatofibrosarcoma protuberans surgery: Experiences of four African surgical oncology units and literature review

“…Surgery is the mainstay of treatment for dermatofibrosarcoma and remains the greatest provider of cure 16,22 . Four surgical procedures have been described in the literature 17,18,22 .…”

“…Surgery is the mainstay of treatment for dermatofibrosarcoma and remains the greatest provider of cure. 16,22 Four surgical procedures have been described in the literature. 17,18,22 These are: resection-suture, resection-healing by secondary intention, resection-flap covering and resection-skin grafting preceded or not by a first phase of healing by secondary intention.…”

“…This localized character gives surgery its place and its role as the main curative treatment 13,14 . Since the description of translocation 17–22, the use of imatinib mysilate has led to improved carcinological results 15,16 . Radiotherapy plays an important role in large, recurrent tumors and marginal resections 14 .…”

“…13,14 Since the description of translocation 17-22, the use of imatinib mysilate has led to improved carcinological results. 15,16 Radiotherapy plays an important role in large, recurrent tumors and marginal resections. 14 Dermatofibrosarcoma has diagnostic and therapeutic peculiarities in the African setting.…”