What is the role of surgery in the treatment of dermatofibrosarcoma protuberans (DFSP)?

Updated: Mar 06, 2020
  • Author: Raman K Madan, MD; Chief Editor: William D James, MD  more...
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Surgical excision remains the mainstay of treatment for dermatofibrosarcoma protuberans (DFSP). [40] Despite controversy, Mohs micrographic surgery has been increasingly accepted as the treatment of choice, while others advocate wide local excision. [13, 34, 41, 42, 43] The fundamental difference of these two techniques is the pathology processing. Usually, the specimen from wide excision is sectioned in conventional bread-loaf fashion, while the Mohs specimen is freshly frozen and sectioned en face along the margins. Mohs surgery requires less tissue removal and allows complete margin assessment. However, large tumor can be a challenge for this very time-consuming procedure.

Because of its infiltrating growth pattern, DFSP commonly extends far beyond the clinical margins; this accounts in part for the high recurrence rate after standard surgical excision. [44] Hence, a wide excision of 2-3 cm or more of the margins beyond clinically identifiable tumor border, down to and including the fascia, is recommended for the treatment of DFSP. [13, 32, 41] Despite wide local excisions, an average recurrence rate of 15.7% is still observed among 1201 body cases and 51.8% is observed among 193 head and neck cases, as reported in the literature since 1951. A superior cure rate (an overall average recurrence rate of 1.3% among 463 cases reported) and tissue conservation are seen when Mohs micrographic surgery is used; thus, Mohs micrographic surgery is now considered the treatment of choice, [45] particularly when a lesion is located in the head and neck region. [18, 41, 46, 47, 48, 49, 50, 51]

Although some Mohs surgeons consider it unnecessary, taking an extra layer of tissue around the surgical defect at the completion of Mohs surgery for permanent pathology section and/or CD34 immunostaining may potentially enhance the cure rate. Alternatively, some have adopted modified Mohs techniques, or so-called "slow Mohs," by using rush paraffin sections instead of a fresh tissue technique. [46, 49, 52, 53, 54] Mohs surgery may not be readily accessible in many parts of the world. The physician should exercise clinical judgment to offer the best treatment available for the patient and consider multidisciplinary collaboration. Studies have demonstrated a low recurrence rate after surgery for DFSP if a multidisciplinary approach and careful pathology margin assessment are used. [55, 56]

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