The Surgical Management of Dermato Fibro Sarcoma Protuberance

Abstract

Background: Dermatofibrosarcoma protuberance is relatively uncommon low to intermediate grade malignant neoplasm with characteristic cytogenetic features. It constitutes less than 0.1% of all malignant neoplasms. Although metastasized rarely, it is locally aggressive tumour with high recurrence rate. It is a one of the rare type of law grade sarcomas that occur s anywhere in the body, usually arise from trunks and extremities. It rarely arises from abdominal wall. Objectives: The main objective of the present study was to study the outcome of surgical treatment of dermatofibrosarcoma protuberance. Methods: This study included 4 patients all retrospective and prospective from September 2008 to December 2013. They were subjected to detailed history taking and examination with relevant investigations and were subjected to surgery. They were followed up for at least 36-month period to assess for any recurrence. Results and Interpretation: Out of 4 patients, all were males. Mean age of presentation was 32 years. Site distribution was 25% trunk, 50% extremities and 25% neck. All patients underwent wide local excision. At the end of follow-up period of up to 3 years after surgery Overall recurrence rate was nil. Because of the potential of local recurrence, therapy for DFSP should be directed toward adequate local excision of the primary lesion. Minimal resection should include a surrounding margin, comprising 3-cm margin of normal skin and removal of underlying deep fascia. Compromising on margins invites higher chances of local recurrence. Conclusion: DFSP behaves like a locally infiltrating neoplasm. Despite their locally aggressive behaviour, distant metastasis occurs with extreme rarity. Because of this, it is reasonable that therapy should be directed towards adequate local excision of the primary lesion. Compromising on margins always invites higher chances of local recurrence. [Bhadreshwara K NJIRM 2014; 5(4):98-101]