Background:
Dermatofibrosarcoma protuberans (DFSP) was first described by Darier and Ferrand in 1924 and then termed DFSP in 1925 by Hoffman. It is a rare, locally aggressive, low-grade fibroblastic mesenchymal tumour. It accounts for only 1% of all soft tissue sarcomas with an annual incidence of 0.8 – 4.5 cases per million. While DFSP most commonly occurs on the trunk, involvement of the breast is rare and there are only a few reported cases in the literature.
Case report:
We report the case of a 39-year-old woman who had a previous breast reduction in her 20s. She presented with a lump in the inferior pole of her right breast. She was reviewed by the breast service and initially, ultrasound showed a 6mm hypoechoic focus superficially and core biopsy suggested spindle cell tumour. However, histopathology of the wider excision confirmed DFSP with positive margins on excision. MRI following this showed post-surgical changes in the breast. After review of the imaging and histopathology in a multi-disciplinary team setting the plan was made for further excision with a view to reconstruct once her tissue returned clear margins. Her past medical history was notable for dysfibrinogenaemia which imposed an increased bleeding risk. This was managed with RiaSTAP and tranexamic acid.
Discussion:
This case report includes a literature review on DFSP in the breast highlighting common demographics, presentations, and treatment. Given its rarity, there is only case series and case report level evidence. The most common presenting complaint is a lump with overlying red or brown-red skin changes. While some cases included treatment with radiotherapy there was no evidence for any associated benefit in routine use.
Conclusion:
DFSP of the breast is a rare presentation. Heightened awareness is important to differentiate it from other breast diseases and surgical excision remains the mainstay of treatment.