Challenging Dermatofibrosarcoma Protuberans- Surgical Management

Abstract

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare, variable grade malignant mesenchymal tumor of the soft tissue, characterized by slow infiltrative growth. It has a high chance of common local recurrence, with rare distant metastases. METHODS We present a retrospective study of twelve patients who were diagnosed with DFSP and operated at our institution in the last 10-year period. We examined the clinicopathological parameters with special emphasis on the margin status regarding the clinical outcome and the follow-up. RESULTS A total of 8 cases underwent re-excision at our institution following primary excision or incisional biopsy performed at a different institution. Seven cases received excision after incisional biopsy at our institution. Five patients developed recurrent disease following primary excision with histological R0 margins at other institutions and received re-excision at our institution. All excisions at our institution resulted in R0 margins with no recurrence recorded at last follow-up (6 to 175; mean 84 months). The mean margin for those who received resection at our institution was 1.67 cm. Negative margins upon primary excision were achieved using a mean margin width of 2.04 cm. Most common tumor localization was the trunk (11 cases). CONCLUSIONS Awareness of this rare entity is important for a prompt diagnosis and a proper management of the disease. The greatest clinical challenge in the management of DFSP is achieving local control. Complete excision of the tumor with surgical margin widths of at least 2 cms is recommended.