Abstract
Usually most patients with dermatofibrosarcoma protuberans (DFSP) may present rather late when the tumor is in protuberant phase due to its rarity and indolent onset. It has a high propensity for local recurrence and destructive nature. Management of DFSP requires a biopsychosocial and Multidisplinary approach regardless of the clinical or immunohistochemical variant. Surgery is the Gold standard management of localized disease. DFSP rarely exhibits any lymphatic or hematogenous dissemination. It is because of its high recurrence rate associated with Wide Local Excision (WLE), the introduction of Mohs micrographic surgery (MMS) has really helped in reducing the rates of recurrence of DFSP. Thus, the aim of this meta-analysis and systemic review is to advocate for MMS over WLE for DFSP and other cutaneous malignancies using DFSP as a prototype. The objective of this study were to conduct a meta-analysis on comparative surgical methods used in the cure of DFSP with regards to WLE verses MMS, to evaluate the cure rates with relation to recurrence rates, offer a recommendation on the various treatment modalities based on the location of lesion, and use of adjuvant therapy in different clinical-medical setups. A comprehensive retrospective analysis search in EMBASE, Google Scholar and Medline (PubMed) for studies published from 2008 to 2018 containing the surgical management of DFSP with WLE verses MMS were reviewed. Five studies of moderate-quality evidence (level B) with a pooled patient load of 684 was analyzed and found for recurrence of DFSP after WLE and MMS to be 9.10% and 2.72% respectively after an average follow-up time for both groups of 5.32 years with a female predominance of 1.58. The trunk is the commonest site for the DFSP lesion which was at 52.80% then the upper and lower extremities zones and the head and neck zones at 31.75% and 15.45% respectively. The pooled adjusted odds ratio (OR) analysis indicated that there was a direct relationship with regards the reduced recurrence rate of DFSP in the MMS group compared to the WLE group (OR:0.31;95%; CI :0.17-0.56). Furthermore, there was significant association between the reduced recurrence rate with the MMS in DFSP patients with a statistical P-value of 0.0001 at 95% CI. The expected increased recurrence rate by zones was in WLE head and neck zone at 38.19% then trunk and extremities zone at 13.34%. In the MMS group it was at of 23.4% as compared to 16.0% in the head and neck zone. Mohs Micrographic Surgery (MMS) is more efficacious in the cure rate and recurrence reduction of DFSP and should be advocated for as first line therapy especially in high recurrence prone zones.
Highlights
Dermatofibrosarcoma protuberans (DFSP) is one of the rare, indolent, spindle cell mesenchymal sarcoma or malignancy of low-grade aggressiveness, that arises in the dermis and can extend to the deep subcutaneous tissue and can affect other under lying structures like muscles and bones in un intervened cases and often stains positive for CD34 [1,2,3]
The eligible studies were articles published between 2008 and 2018, and had sample sizes range of 48 to 284.Our study showed a female predominance of 1.58 in a population study of 684 with an average age group of the patients being 44.6 years for both categories of wide local excision (WLE) and those of Mohs microscopic surgery (MMS)
The pooled adjusted odds ratio (OR) analysis, indicated that there was a direct relationship with regards the reduced recurrence rate of DFSP in the Mohs micrographic surgery (MMS) group compared to the WLE group (OR: 0.31;95% confidence interval (CI) :0.17-0.56) as shown in Table 3 [5759]
Summary
Dermatofibrosarcoma protuberans (DFSP) is one of the rare, indolent, spindle cell mesenchymal sarcoma or malignancy of low-grade aggressiveness, that arises in the dermis and can extend to the deep subcutaneous tissue and can affect other under lying structures like muscles and bones in un intervened cases and often stains positive for CD34 [1,2,3]. It was first described by Darier and Ferrand Hoffman officially coined the term dermatofibrosarcoma protuberans [4]. These tumors have been associated with chromosomal translocation at position 17:22 leading to an overexpression of tyrosine kinase PDGFB, which can be targeted with Imatinib, a tyrosine kinase inhibitor [12]
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