Surgical Treatment of Dermatofibrosarcoma Protuberans

Abstract

Dermatofibrosarcoma protuberans is a rare dermal tumor that recurs after inadequate primary treatment. In a retrospective study, we analyzed the outcomes of 117 patients (mean age 39 years) treated surgically for a dermatofibrosarcoma protuberans. In most cases (107 patients), surgery was performed according to a protocol of taking wide peripheral resection margins of 5 cm and by resecting a disease-free anatomic zone deep to the lesion. The mean follow-up was 61 months. The results suggest a difference in prognosis between patients treated primarily with wide initial resection and those referred secondarily with recurrent disease following previous treatment by narrow resection margins. There was no recurrent disease in the 66 patients treated primarily by wide peripheral (5 cm) and deep resection of the tumor. Of the 41 patients referred secondarily at the time of recurrence, 2 developed further local disease within a year, despite equally aggressive local treatments. Both patients eventually died of metastatic fibrosarcoma. We emphasize the value of a highly aggressive local resection in the primary treatment of dermatofibrosarcoma protuberans to minimize local recurrence and potential malignant transformation. Modern reconstructive techniques provide satisfactory solutions for defects of almost any size and composition. Our findings suggest that radical primary resection of dermatofibrosarcoma protuberans in conjunction with immediate reconstruction of the primary defect provides the best outcome for the patient.

Reconstruction of a Scalp and Skull Defect with Free Latissimus Dorsi Myocutaneous Flap following Dermatofibrosarcoma Protuberans

“…Dermatofibrosarcoma protuberans is classified as a fibrohistocytic tumour of intermediate-grade malignancy. 4 Dermatofibrosarcoma protuberans is reported to exhibit frequent local recurrence [1][2][3] but to rarely exhibit metastasis. 4 Simple excision of the tumour was initially performed on this patient, but frequent local recurrence Figure 1 Artificial dura mater (arrow) was exposed due to the skin defect following surgery.…”

“…Dermatofibrosarcoma protuberans must be excised along with the skin with a 5 cm margin, 1 thus resulting in an extensive scalp defect. After wide excision of dermatofibrosarcoma protuberans, reconstruction with a latissimus dorsi myocutaneous flap should be the first choice because it permits the harvesting of a wide myocutaneous flap that includes a long and thick vascular pedicle.…”

“…1,2 Dermatofibrosarcoma protuberans tends to occur on the trunk and proximal region of the extremities while it rarely develops on the head. It frequently recurs locally due to incomplete excision [1][2][3] but rarely metastases.…”

Low recurrence rate after surgery for dermatofibrosarcoma protuberans

“…1 Wide excision with histologically negative margins is the cornerstone of treatment, but relatively high recurrence rates are described in the literature, [1][2][3][4] especially for DFSP lesions located on the head and neck. 5,6 Despite the achievement of "negative" margins, it is believed that the high recurrence rate is due to the fact that large portions of the true margins are not evaluated by standard histologic processing, combined with the difficulty identifying the occult, fingerlike projections that are characteristic of DFSP and are responsible for tumor recurrence.…”

Distant Metastasis of Dermatofibrosarcoma Protuberans of the Scalp-Case Report-

“…9) The optimum therapy for DFSP is initial wide local resection. Wide resection of more than 5 cm from the margin of the tumor 1) and Mohs micrographic surgery should be performed. 2,15) The risk of local recurrence correlates well with the extent of the wide excision.…”