Dermatofibrosarcoma Protuberans: One Centre Experience

Abstract

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue tumor that is classified as a non-melanoma skin cancer. Surgical excision with wide safety margins is the cornerstone treatment and frequently requires advanced reconstruction methods for wound closure. This study aimed to report our experience with the management of seven consecutive patients with dermatofibrosarcoma protuberans. Materials and Methods: We performed a retrospective study of seven consecutive patients with dermatofibrosarcoma protuberans admitted to our Plastic and Reconstructive Surgery Department of Clinical Emergency Hospital “Prof. Dr. Agrippa Ionescu” Bucharest, Romania, between July 12, 2018, and July 4, 2022. Results: The mean age of the patients was 38.2 years. In 4 patients, the primary treatment consisted of wide local excision, while 3 presented to our clinic for re-resection. All cases required complex reconstruction methods: 3 patients with a split-thickness skin graft (STSG), 3 patients with local flaps, and in one patient, because of the superior size of the post-excisional defect, we used local advancement flaps and STSG. In two cases, the histopathological diagnosis revealed fibrosarcomatous differentiation (the largest primary tumor dimension), one of which showed fascial invasion, and both of them received adjuvant radiotherapy. Conclusion: A soft tissue tumor with a clinical appearance suggestive of DFSP, especially in cases of increased tumor sizes, incisional biopsy and subsequent multidisciplinary approach will lead to a wide surgical excision with negative histopathological margins, as a standard treatment, for a low-grade sarcoma, which rarely metastasizes.