A 10‐year review of surgical management of dermatofibrosarcoma protuberans*

Abstract

Summary Background Dermatofibrosarcoma protuberans (DFSP) is a rare skin cancer. Standard treatment in the UK is either wide local excision (WLE) or Mohs micrographic surgery (MMS). It is unclear which approach has the lower recurrence rate. Objectives We undertook a retrospective comparative review of surgical management of DFSP in the UK National Health Service in order to define (i) current surgical practice for primary and recurrent DFSP, (ii) local recurrence rates for primary DFSP and (iii) survival outcomes for DFSP. Methods A retrospective clinical case‐note review of patients with histologically confirmed DFSP (January 2004 to December 2013) who have undergone surgical treatment. Results The surgical management of 483 primary and 64 recurrent DFSP in 11 plastic surgery and 15 dermatology departments was analysed. Almost 75% of primary DFSP (n = 362) were treated with WLE and 20% (n = 97) with MMS. For recurrent DFSP, 69% (n = 44) and 23% (n = 15) of patients underwent WLE and MMS, respectively. Recurrent primary DFSP occurred in six patients after WLE and none after MMS. The median follow‐up time was 25·5 months (interquartile range 6·8–45·1) for new and 19·8 (IQR 4·5–44·5) for recurrent DFSP [Correction added on 1 Feb 2021, after first online publication: 4.8 years (interquartile range 3.5‐5.8) was incorrect], with eight reported deaths during the follow‐up analysis period (one confirmed to be DFSP related). Conclusions WLE was the most common surgical modality used to treat DFSP across the UK. The local recurrence rate was very low, occurring only after WLE. Although a prospective randomized controlled trial may provide more definitive outcomes, in the absence of a clearly superior surgical modality, treatment decisions should be based on patient preference, clinical expertise and cost.

Dermatofibrosarcoma protuberans: slowly gathering evidence for optimal treatment of a slow‐growing tumour

“…Almost 20% of standard excisions were incomplete at the first attempt. Recurrence rates in the current study were lower than previously reported (1.2% vs. 7%) 1,4 . However, this is probably an underestimation as the median follow‐up time of treated DFSPs in this study was very short (2.2 years).…”

“…Recurrence rates in the current study were lower than previously reported (1.2% vs. 7%). 1,4 However, this is probably an underestimation as the median follow-up time of treated DFSPs in this study was very short (2.2 years). The Dutch study showed that half of all recurrences occurred after 2 years.…”

“…The majority of all patients in the study of Durack et al . were treated with wide local excision (WLE) and around 25% were treated with MMS 1 . The authors found only six recurrences, all in the excision group and none in the patients treated with MMS.…”

“…In this issue of the BJD, Durack et al 1 report on the surgical management of dermatofibrosarcoma protuberans (DFSP), a rare and slow-growing soft tissue tumour. [1][2][3] They went to a great effort to review the charts of almost 500 cases of DFSP across the UK. DFSP rarely metastasizes but due to its tentaclelike invasion, lesions are larger than clinically suspected, leading to high recurrence rates after treatment.…”

Mohs micrographic surgery in dermatofibrosarcoma protuberans: Rate and risk factors for recurrence in a prospective cohort study from the Spanish Registry of Mohs Surgery (REGESMOHS) and review of the literature

“…Durack et al 16 performed a retrospective comparative review of the surgical management of DFSP in the United Kingdom National Health Service in order to define the local recurrence rate for primary tumors and survival outcomes for DFSP. 483 primary and 64 recurrent DFSP was included; 75% of primary tumors were treated with WLE and 20.1% with MMS.…”