Frozen Section Mohs Micrographic Surgery for Dermatofibrosarcoma Protuberans-A Case Series of 38 Patients in NSW, Australia.

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.

Dermatofibrosarcoma protuberans: Wide local excision vs. Mohs micrographic surgery

Dermatofibrosarcoma protuberans (DFSP) is a rare, malignant skin cancer commonly found on the trunk. DFSP includes primary DFSP and Bednar tumour, a pigmented, intermediate-grade subtype of primary DFSP that has an increased tendency for local invasion and secondary recurrence. DFSP can be surgically resected with Mohs micrographic surgery (MMS) or wide local excision (WLE). Despite MMS being considered the ‘gold standard’ for the treatment of several malignant skin cancers, WLE is frequently used to treat DFSP, and the difference in outcomes has not been well explored. To fill in this literature gap, data from the National Cancer Database were analysed to explore survival based on surgical modality. A retrospective cohort study of 8878 patients diagnosed with primary DFSP (8699) and Bednar tumours (179) between 2004 and 2021 was conducted across 1500 Commission on Cancer facilities located in 49 US states and Puerto Rico. The impact of surgical treatment type on survival was analysed by Kaplan–Meier survival analysis. Other demographic factors, such as age, sex, race and comorbidity were considered to determine any trends in our cohort. In total, 5090 patients with DFSP had survival data based on surgery type, with 805 undergoing MMS and 4285 undergoing WLE. Overall, 53.7% of patients were female. With respect to race, 70.3% of patients were White, 21.7% Black, 4.3% Asian and 3.7% other. The average age of our cohort was 43.2 years (SD 15.7, range 0–90). Lastly, > 90% of the patients had a Charlson–Deyo comorbidity score of zero and were relatively healthy. MMS was associated with significantly increased survival compared with WLE (P = 0.012), with patients living approximately 6 months longer than those treated with WLE (210 months vs. 204 months, respectively). While the standard of practice for DFSP is general surgical excision, including WLE, this study supports MMS as the preferred treatment based on improved survival outcomes. Although there are many factors that may require non-MMS surgery, these results highlight the importance of increased access to a variety of surgical treatment options for all patients. Furthermore, they emphasize the need for more clinical data on DFSP to better equip physicians to diagnose and treat this rare but dangerous skin cancer. Especially in comparison with the average age of skin cancer diagnosis at 70 years, six additional months of survival becomes increasingly important for the young and healthy patient population affected by DFSP.

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma with a propensity for recurrence. Its management, particularly in the head and neck (H&N) region, presents unique challenges. This study aimed to evaluate the effectiveness of Mohs micrographic surgery (MMS) compared to wide local excision (WLE) in treating H&N DFSP and its impact on recurrence rates and tissue preservation. A comprehensive search was conducted in PubMed/MEDLINE, yielding 29 relevant studies. We included studies comparing MMS and WLE in adult patients with H&N DFSP and reporting local recurrence outcomes. Data were analyzed using random effects analysis, with a meta-analysis performed for comparative studies. Analysis of studies demonstrated a lower recurrence for MMS. Comparative analysis of five studies involving 117 patients showed a significantly lower recurrence rate in the MMS group (2%) compared to the WLE group (19%). Margin status varied between studies, with some achieving negative margins at shorter distances. In the management of H&N DFSP, MMS has emerged as a superior surgical technique, consistently associated with reduced recurrence rates and the potential for tissue preservation. The adoption of MMS should be considered for its capacity to achieve negative margins with fewer processing steps, particularly in anatomically complex regions like the H&N.

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. 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. A retrospective clinical case-note review of patients with histologically confirmed DFSP (January 2004 to December 2013) who have undergone surgical treatment. 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). 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.

Local recurrence (LR) rates of dermatofibrosarcoma protuberans (DFSP) treated with different surgical modalities are unknown. To evaluate the differences in LR rates of DFSP treated with wide local excision (WLE) versus Mohs micrographic surgery (MMS). Pertinent studies of DFSP treated with either WLE or MMS were identified through a search of multiple databases, including Ovid MEDLINE (1946-2018), Embase (1988-2018), Web of Science (1975-2018), and Scopus (1970-2018). Comparative 2-arm and noncomparative single-arm studies were assessed through meta-analyses. Of the 517 studies identified, 88 met inclusion criteria (12 comparative studies; 76 single-arm studies). In the 12 comparative studies, 352 patients with DFSP underwent MMS and 777 patients with DFSP underwent WLE. The LR rate was 1.7% after MMS and 3.7% after WLE (odds ratio, 1.549; 95% CI, 0.710-3.381; p = .27). In the 76 noncomparative studies, 980 patients underwent MMS (LR rate, 1.5%; 95% CI, 0.9%-2.1%; p < .001), and 2,215 patients underwent WLE (LR rate, 9.4%; 95% CI, 7.5%-11.3%; p < .001). The LR rate of DFSP in patients treated with MMS is lower than in patients treated with WLE. Because of high rates of postoperative DFSP LR, MMS should be strongly considered when available.

Dermatofibrosarcoma protuberans (DFSP) is a rare borderline malignant or potentially low-grade malignant neoplasm with a high local recurrence rate and a low risk of metastasis. DFSP usually occurs on the trunk in people aged 20-50 years, and is characterized by multiple indurated nodules varying in size and arising on atrophic plaques. Histologically, the tumor consists of uniform spindle cells which grow infiltratively and are arranged in a storiform or cartwheel pattern. The standard therapy of DFSP is wide local excision with a margin of 2-3 cm. Mohs' surgery and modified Mohs' surgery have shown a good prospect in the treatment of DFSP. Radiation therapy can serve as an adjuvant treatment after surgery. In recent years, the pathogenesis of DFSP has been further clarified and studies have shown that chromosomes 17 and 22 are rearranged in 90% of patients with DFSP. As a targeted drug, imatinib has been approved for the treatment of DFSP in patients with unresectable, metastatic and/or recurrent DFSP. Key words: Dermatofibrosarcoma; Surgical procedures, operative; Mohs surgery; Radiotherapy dosage; Molecular targeted therapy

Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma with a propensity for local recurrence. Treatments with wide excision, Mohs surgery, and other approaches have been reported with widely variable local control rates. The objective of this study was to review the experience with a multidisciplinary approach employing wide excision and Mohs surgery selectively in the treatment of patients with DFSP at a single academic institution over the past 10 years. The records of 62 patients with 63 DFSP tumors who underwent wide excision, Mohs surgery, or a multidisciplinary combination approach from January 1991 to December 2000 were reviewed retrospectively. Primary endpoints included the ability to extirpate the DFSP lesion completely, the tumor recurrence rate, and the need for skin grafts or local tissue flaps. Additional objectives included defining surgical practice patterns at the authors' institution. Sixty-three DFSP lesions were removed from 62 patients. At a median follow-up of 4.4 years, no local or distant recurrences were detected in any patient. Forty-three lesions were treated with wide local excision, 11 lesions were treated with Mohs surgery, and 9 lesions were treated with a combination approach. Ninety-five percent of lesions that were approached initially with wide local excision were cleared histologically. Two patients (5%) received postoperative radiation for positive margins after undergoing maximal excision. Eighty-five percent of lesions that were approached initially with Mohs surgery were cleared histologically. The remaining 15% of lesions subsequently were cleared surgically with a wide excision. DFSP lesions that were approached initially with Mohs surgery tended to be smaller. Patients with head and neck lesions most often underwent Mohs surgery or were treated with a multidisciplinary combination approach (87%). Wide local excision with careful pathologic analysis of margins was found to have a very low recurrence rate and was used for the majority of patients with DFSP lesions at the authors' institution. Wide local excision, Mohs surgery, and a multidisciplinary combination approach, selected based on both tumor and patient factors, were capable of achieving very high local control rates in the treatment of DFSP. The evolution of a multidisciplinary approach has provided a level of expertise that no single individual could achieve for the treatment of the full spectrum of DFSP lesions at the authors' institution.

Treatment of dermatofibrosarcoma of the head and neck with Mohs surgery with paraffin sections

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing skin cancer that is primarily treated with surgery. Because this cancer can spread deep into the skin and surrounding tissue in an irregular way, it is notoriously difficult to ensure that it has been completely removed after conventional surgery and histological examination. Standard treatment in the UK is either wide local excision (WLE) or Mohs micrographic surgery (MMS). In WLE, the tumour and a predetermined margin of skin around it is removed. The margin is usually in the range of 1-5 cm. MMS involves removing the tumour and a much smaller margin around it, followed by immediate microscopic examination of the edges of the removed skin. Areas still containing tumour cells are then removed. This is repeated until all the edges are clear of tumour cells. This method allows the surgeon to track the tumour and only remove tissue where is it present. This study was carried out at several centres in the UK. Clinical records were examined of cases of DFSP between 1 January 2004 and 31 December 2013. This study includes the largest number of cases of DFSP reported in the UK: 483 new and 64 recurring cases of DFSP, in 11 plastic surgery and 15 dermatology departments. The methods used to perform these 547 surgeries were analysed. Almost 75% of cases of new DFSP were treated with WLE and 20% were treated with MMS. For recurring DFSP, 69% of patients had WLE and 23% had MMS. After a median follow-up period of just over 2 years for new DFSP and 1.6 years for recurring DFSP, the tumour returned in six patients after WLE and none after MMS. Although no DFSP were found to have returned after MMS, the study could not detect any significant difference in recurrences between the two surgical methods. Until data from more detailed studies are available, the choice of treatment for DFSP should be based on the patient's preference, treatment availability and cost. Linked Article:Durack et al. Br J Dermatol 2021; 184:731-739.

Introduction. Dermatofibrosarcoma Protuberans (DFSP) is a rare cutaneous tumour of low/intermediate malignant potential, which occasionally arises on the vulva. Historically, the treatment has been wide local excision (WLE). Mohs micrographic surgery (MMS) is now recommended to ensure precise margin control. MMS to treat DFSP of the trunk and extremities is well documented. However, no report to date has described its use in vulval DFSP. Case History. A 39 year old woman presented with a longstanding nodule in the left labium majus. Histology after surgical removal showed an incompletely excised DFSP. MMS was undertaken with primary closure of the defect. Three years following treatment there is no evidence of recurrence. Discussion. The local recurrence rate of DFSP after WLE ranges from 0–75%. Finger-like projections from DFSP into surrounding tissue often results in incomplete excision. Representative vertical sections used in WLE assess less than 1% of the total tumour margin. MMS uses systematic horizontal sectioning. 100% of the tumour margin is microscopically examined. MMS is now advocated to ensure precise margin control.

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue tumor with slow infiltrative growth and local recurrence if inadequately excised. To compare long-term outcomes after Mohs micrographic surgery (MMS) and wide local excision (WLE). Records of patients with DFSP surgically treated with WLE or MMS from January 1955 through March 2012 were retrospectively reviewed. Mean follow-up for patients treated with MMS (n = 67) and WLE (n = 91) was 4.8 and 5.7 years, respectively. Twenty-eight patients (30.8%) with WLE had recurrence (mean, 4.4 years), whereas only 2 (3.0%) with MMS had recurrence (1.0 and 2.6 years). Recurrence-free survival rates at 1, 5, 10, and 15 years were significantly higher with MMS (p < .001). Mean preoperative lesion sizes were similar (5-6 cm) between the 2 groups, whereas mean (standard deviation) postoperative defect sizes were 10.7 (4.3) cm and 8.8 (5.5) cm for WLE and MMS, respectively (p = .004). Primary closure was used for 73% of MMS cases, whereas WLE more commonly used flaps, grafts, or other closures (52%). Two Mohs layers typically were required for margin control. Surgical excision with meticulous histologic evaluation of all surgical margins is needed for DFSP treatment to achieve long-term high cure rates and low morbidity.

Dermatofibrosarcoma protuberans (DFSP) may have a deceptively benign clinical appearance, including a nonprotuberant presentation. Patients with DFSP often perceive misdiagnoses and delays in receiving a diagnosis. Use of existing, patient-designed Facebook patient support groups (FBSGs) to recruit large numbers of patients with rare diseases may be an effective novel research method. To collaborate with patients with rare disease through social media and answer questions important to both patients and the medical field, including sources of diagnostic delay, risk of recurrence, and flat presentation of DFSP. A multiple-choice survey created by a team of medical practitioners and patients with DFSP was administered to 214 patients with DFSP or family members from international DFSP FBSGs and a nonprofit foundation patient database via Lime Survey from October 30 to November 20, 2015. The survey asked questions designed to determine risk of recurrence and metastasis, surgical outcomes, sources of diagnostic delay, symptoms of recurrence, number of recurrences, scar size, and number of clinicians seen before biopsy. Statistical analysis was performed from January 1, 2016, to April 1, 2019. The study goal was to collect at least 200 survey responses. Of 214 survey respondents (169 females and 45 males; mean [SD] age, 40.7 [12.1] years; range, <1 to 72 years), 199 were patients with DFSP and 15 were family members. Delays occurred between the patient noticing the DFSP lesion and receiving a diagnosis of DFSP (median, 4 years; range, <1 to 42 years). Most patients (112 [52.3%]) believed that they received a misdiagnosis at some point: by dermatologists (35 of 107 [32.7%]), primary care clinicians (80 of 107 [74.8%]), or another type of physician (27 of 107 [25.2%]). The most frequent prebiopsy clinical suspicion included cyst (101 [47.2%]), lipoma (30 [14.0%]), and scar (17 [7.9%]). Many patients first noticed their DFSP as a flat plaque (87 of 194 [44.8%]). Of these lesions, 73.6% (64 of 87) became protuberant eventually. Surgical treatments included Mohs micrographic surgery (56 of 194 [28.9%]), wide local excision (122 of 194 [62.9%]), and conservative excision (16 of 194 [8.2%]). The reported rate of recurrence was 5.4% (3 of 56) for Mohs micrographic surgery, 7.4% (9 of 122) for wide local excision, and 37.5% (6 of 16) for conservative excision. The higher rate of recurrence for conservative excision was significant (P = .001); there was no significant difference in the rate of recurrence between Mohs micrographic surgery and wide local excision (P = .76). This study reports what appears to be disease-relevant statistics from the largest survey of patients with DFSP to date. Because of the dissonance between the name of the neoplasm and its clinical presentation, the alternative term dermatofibrosarcoma, often protuberant is proposed. This study suggests that FBSGs are useful tools in medical research, providing rapid access to large numbers of patients with rare diseases and enabling synergistic collaborations between patients and medical researchers.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous and subcutaneous mesenchymal malignant tumor characterized by high rates of local recurrence and low risk of metastasis. Histopathological and immumohistochemical study can be used to diagnose DFSP. Recent studies have shown that cell genetics and molecular biology, imagiology, dermatoscopy and cutaneous computed tomography (reflectance confocal microscopy) can also play an important auxiliary role in the diagnosis, treatment and evaluation of DFSP. At present, the surgical treatment of DFSP mainly includes wide local excision (WLE) and Mohs micrographic surgery (MMS) . Each of the two approaches has its advantages and disadvantages. After surgery, radiation therapy and comprehensive immunological treatment can serve as adjuvant treatment. Cryotherapy is effective in patients with recurrent DFSP, and the targeted agent imatinib can be combined for inoperable patients or patients with positive surgical margins. In addition, antiestrogenic drugs (such as tamoxifen) or the new targeted agent heparanase inhibitor roneparstat (SST0001) may have some therapeutic effects. Key words: Dermatofibrosarcoma; Pathology, clinical; Diagnosis; Mohs surgery; Therapeutic Uses

Although advances have been made in the understanding of recurrence patterns in dermatofibrosarcoma protuberans, the current understanding of disease-specific mortality after surgical management is limited. To understand disease-specific mortality rates associated with dermatofibrosarcoma protuberans treated with wide local excision (WLE) versus Mohs micrographic surgery (MMS). A systematic literature search was conducted on March 6, 2023, to identify patients treated with MMS or WLE for dermatofibrosarcoma protuberans. A total of 136 studies met inclusion criteria. Overall, the disease-specific mortality rate was not significantly different after treatment with MMS (0.7%, confidence interval [CI] 0.1-1.2, p : 0.016) versus WLE (0.9%, CI 0.6-1.2, p < .001). For recurrent tumors, the MMS treatment group had a statistically significantly lower disease-specific mortality rate (1.0%, CI 0.0-2.0, p 0.046) compared with the WLE treatment group (3.5%, CI 2.0-5.1, p < .001). The mean follow-up for all studies was 57.6 months. The authors' meta-analysis suggests there is no substantial difference in disease-specific mortality between MMS and WLE in patients with dermatofibrosarcoma protuberans, except in the case of recurrent tumors, where MMS seems to confer a survival advantage.