High-frequency ultrasound-assisted Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a superficial sarcoma characterized by infiltrative growth with tentacle-like borders. Mohs micrographic surgery (MMS) is the preferred treatment option for DFSP. However, the imprecise boundary localization in MMS leads to an increased number of Mohs layers required and a longer surgery time. High-frequency ultrasound has excellent tissue recognition capability for DFSP, allowing for precise boundary marking. MATERIALS AND METHODS: In this study, we retrospectively analyzed 14 cases of DFSP treated with MMS using preoperative ultrasound localization and three-dimensional reconstruction at Xiangya Hospital over the past 5 years. We also reviewed previous studies on MMS for DFSP treatment. RESULTS: It was found that the average number of Mohs layers for patients after preoperative ultrasound localization was 1.57, ranging from 1 to 3, which was less than the previously reported 1.86 layers, ranging from 1 to 12. This effectively reduced the number of Mohs layers required. CONCLUSIONS: By utilizing preoperative high-frequency ultrasound to determine the boundaries and depth of DFSP, the number of Mohs layers can be effectively reduced, leading to less workload for pathological examination, shorter operation time, and reduced surgical risks for patients. Ultrasound imaging data can be used for three-dimensional reconstruction, enabling less experienced Mohs surgeons to have a visual understanding of the morphology and extent of infiltration of the lesions. This aids in developing optimal surgical plans, smoothing the learning curve, and promoting the wider adoption of MMS.

Dermatofibrosarcoma Protuberans in "Slow Mohs-tion": Multidisciplinary Approach of A Pediatric Dermatofibrosarcoma Protuberans of the Scalp with Slow Mohs Micrographic Surgery and A Double Rotational-Advancement Scalp Flap.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft-tissue sarcoma with primary incidence of 4.1 per million person-years and accounts for 0.1% of all malignancies. In patients under the age of 19, DFSP comprises 6% of cases with an annual incidence of 1 in 1,000,000. It is a slow-growing malignancy with low metastatic potential. However, DFSP is notable for its high rates of local recurrence due to local invasion with its classic "finger-like" projections into normal tissue. We discuss a case of dermatofibrosarcoma protuberans on the scalp of a 14-year-old male with delayed diagnosis, which required extensive resection through slow Mohs Micrographic Surgery (sMMS). This resection created a sizeable scalp defect of nearly 100 cm 2 , which mandated creative reconstruction using a novel double rotational-advancement scalp flap to close the defect while maintaining the patient's hairline for optimal cosmesis.

Analysis of surgical margins and prognostic factors in dermatofibrosarcoma protuberans after wide local excision: A multicenter study of 116 Japanese patients.

Cutaneous dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor characterized by a high risk of local recurrence but a low risk of metastasis. Wide local excision (WLE) has been an important treatment option, but its clinical outcomes and safety have not been thoroughly evaluated in previous reports. The aim of this study was to determine appropriate surgical margins (deep and lateral) and prognostic factors associated with recurrence-free survival (RFS) of DFSP. A database collected by two dermatology departments in Japan was retrospectively reviewed to identify 116 patients with DFSP who underwent complete resection with WLE between 1994 and 2021. Sixty-one men (53%) and 55 women (47%) were included in our cohort. The primary sites of DFSP were as follows: 11 head and neck (9%); seven face (7%); 12 upper extremities (10%); 20 lower extremities (17%); and 66 trunk (57%). There were 103 cases (89%) of primary DFSP and 13 cases (11%) of recurrent DFSP. Total 10-year RFS was 96.6%. There were significant differences in RFS by tumor size (median size: 3 cm), disease status (primary versus recurrent DFSP), and fibrosarcomatous change (positive versus negative) (all p < 0.05). Two patients (1.7%) with buccal or head lesions had positive deep margins. In all cases, the lateral margin was negative at the postoperative evaluation. Tumor size, disease status, and fibrosarcomatous change are important risk factors for recurrence. Both face and head-neck lesions were more likely to have positive deep margins than other anatomic areas in DFSP. Although this study was limited by its retrospective design, a narrow 2-cm lateral margin is especially considered for low-risk patients.

Dermatofibrosarcoma protuberans of the breast.

Dermatofibrosarcoma protuberans (DFSP) of the breast is an infrequent soft tissue sarcoma that usually affects young to middle-aged women. Our case report describes a unique occurrence of DFSP of the breast in an adolescent girl, which was initially being managed as a keloid for 2 years under dermatology despite being refractory to treatment. Once the diagnosis of DFSP was confirmed through punch biopsy, our patient underwent surgical excision of the lesion under general anaesthesia. Our patient was at an increased risk of damage to the ductal system due to proximity of the lesion to the nipple-areolar complex, warranting the need for early recognition and treatment. As demonstrated by our case, DFSP of the breast can be difficult to diagnose since it resembles a range of benign and malignant pathologies of the breast.

Conventional and contrast-enhanced ultrasound in the differential diagnosis of recurrent dermatofibrosarcoma protuberans and postoperative scar.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) has a high recurrence rate after resection. Because of the lack of specific manifestations, recurrent DFSP is easily misdiagnosed as post-resection scar. A few series have reported ultrasound findings of recurrent DFSP; moreover, the usefulness of contrast-enhanced ultrasound in differentiating recurrent DFSP has not been studied. OBJECTIVE: We investigated conventional and contrast-enhanced ultrasound in the differential diagnosis of recurrent DFSP and post-resection scar. METHODS: We retrospectively evaluated the findings of conventional and contrast-enhanced ultrasound in 34 cases of recurrent DFSP and 38 postoperative scars examined between January 2018 and December 2022. RESULTS: The depth and vascular density of recurrent DFSP were greater than those of postoperative scars (P < 0.05). On gray-scale ultrasound, recurrent DFSP lesions were more commonly irregular, heterogeneous, and hypoechoic, with finger-like projections and ill-defined borders. Postoperative scar was more likely to appear as hypoechoic and homogeneous with well-defined borders (P < 0.05). On color Doppler ultrasound, recurrent DFSP was more likely to feature rich arterial and venous blood flow, and postoperative scar was more likely to display poor blood flow (P < 0.05). On contrast-enhanced ultrasound, recurrent DFSP was more likely to feature heterogeneous hyper-enhancement, and postoperative scar was more likely to display homogeneous iso-enhancement (P < 0.05). Recurrent DFSP presented a higher peak and sharpness than postoperative scar (P < 0.05). CONCLUSION: Conventional and contrast-enhanced ultrasound produced distinct features of recurrent DFSP and post-resection scar, which could improve the accuracy of differential diagnosis.

Review on the Role of Paraffin-embedded Margin-controlled Mohs Micrographic Surgery to Treat Skin Tumors.

Paraffin-embedded margin-controlled Mohs micrographic surgery (PMMS) includes various procedures such as slow Mohs or deferred Mohs technique, the Muffin and Tübingen techniques, and staged margin excision, or the spaghetti technique. PMMS is a variation of conventional Mohs micrographic surgery (MMS) that allows histopathological examination with delayed margin control. PMMS requires minimum training and may be adopted by any hospital. The setback is that PMMS can require procedures across multiple days. PMMS lowers the rate of recurrence of basal cell carcinoma vs wide local excision in high-risk basal cell carcinoma, and improves the rates of recurrence and survival in lentigo maligna. PMMS can be very useful in high-risk squamous cell carcinoma treatment. Finally, it is a promising technique to treat infrequent skin neoplasms, such as dermatofibrosarcoma protuberans, or extramammary Paget's disease, among others. In this article, we present a literature narrative review on PMMS, describing techniques and indications, and highlighting long-term outcomes.

Disease-Specific Mortality of Dermatofibrosarcoma Protuberans After Mohs Surgery Versus Wide Local Excision: A Systematic Review and Meta-Analysis.

BACKGROUND: 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. OBJECTIVE: To understand disease-specific mortality rates associated with dermatofibrosarcoma protuberans treated with wide local excision (WLE) versus Mohs micrographic surgery (MMS). MATERIALS AND METHODS: A systematic literature search was conducted on March 6, 2023, to identify patients treated with MMS or WLE for dermatofibrosarcoma protuberans. RESULTS: 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. CONCLUSION AND RELEVANCE: 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.

Subtotal Thigh Flap for Large Abdominal Wall Defect Reconstruction in Patient With Recurrent Dermatofibrosarcoma Protuberans: A Case Report and Literature Review.

BACKGROUND: Harvesting an adequate-sized flap is challenging for reconstructing large defects on the abdominal wall. A subtotal thigh flap would be one of the choices as it provides a well-vascularized large flap with muscle components. Moreover, dermatofibrosarcoma protuberans (DFSP) is a low-grade dermal neoplasm with a high recurrence rate. There is still no consensus on the extent of resection to prevent a recurrence. OBJECTIVES: We present a case of a patient who underwent the reconstruction of a large abdominal wall defect with a subtotal thigh flap after the resection of recurrent DFSP. MATERIALS AND METHODS: A 59-year-old man killed from a recurrent huge mass in the lower abdomen with an invasion of the small intestine. His baseline characteristics and records of operations, medications, and outcomes were reviewed. RESULT: After tumor excision, a 28 × 30-cm subtotal thigh flap was harvested from his left thigh to reconstruct the abdominal defect. A microvascular anastomosis with left deep inferior epigastric vessels was made eventually. The flap was in good condition, and the donor site was covered with a split-thickness skin graft. CONCLUSIONS: Subtotal thigh flap may be considered for large abdominal wall defect reconstruction as it allows good perfusion of relatively large skin paddles compared with other free flaps. Also, patients with DFSP need definite margin-free resection and close follow-up to prevent a recurrence.

Novel role of fluorescent in situ hybridization technique (FISH) in recommended surgical margins of dermatofibrosarcoma protuberans: A preliminary study.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor. There is no standard recommendation for its surgical management. The currently used histological analysis are HES (hematoxylin eosin saffron) staining and immunohistochemistry for CD34 expression in particular cases. Fluorescent in situ hybridization (FISH) technique is only used to qualify the DFSP as translocated or non-translocated and is not used as a diagnostic method. The aim of our study was to determine by FISH (as a diagnostic method) whether cancerous cells that could not be identified through HES staining±immunohistochemistry were present at the two-centimeter margins that were found to be tumor-free. METHODS: Samples from patients who underwent surgery between 2010 and 2018 were collected. Intralesional and peripheral (at 2cm margins) paraffin slides were included. An average of 7.4 slides per specimen was analyzed. Firstly, the preselected slides were reread by a senior pathologist to confirm the absence of microscopic findings of DFSP at 2cm margins. Secondly a FISH analysis was used as a quantitative diagnostic approach, in order to find the t(17;22) translocation. RESULTS: Among the seven specimens that included 2cm margins, two samples presented one or more translocations, which were not visible in standard morphology assessments at two centimeters tumor-free margins. CONCLUSIONS: FISH analysis can have a new role in defining tumor-free margins. This would reduce the incidence of disease recurrence after resection and improve the post-operative complementary care.

[Fascio-cutaneous exeresis of a dermatofibrosarcoma of Darier-Ferrand of the right flank]. / Exérèse fascio-cutanée d'un dermatofibrosarcome de Darier-Ferrand du flanc droit.

INTRODUCTION: Darrier-Ferrand dermatofibrosarcoma (DFSC) is the most common cutaneous sarcoma. It generally affects subjects with an average age of 40 years, without gender or race predominance. It is a tumour characterised by a slow evolution and local aggressiveness. The reasons for consultation are pain, pruritus or rapidly progressive evolution. There are no specific imaging studies for this tumour. The diagnosis of certainty is based on immunohistochemistry with positive CD34 labelling. Treatment is surgical based on wide excision. MATERIAL AND METHODS: This is an observation of a patient operated in our department for the initial diagnosis of cutaneous leiomyosarcoma of the right flank that rapidly increased in volume to 10cm in six months. A large fasciocutaneous excision was performed. The postoperative course was simple. DISCUSSION: In our patient, this lesion occurred on an old burn scar. This notion of skin trauma preceding the appearance of DFSC is reported in 10 to 20% of cases. The rapid increase in volume was the reason for consultation. The diagnosis of DFSC could only be made on definitive analysis of the surgical specimen, which showed positive immunostaining for CD34. The occurrence of metastases, although rare, confers a survival of no more than two years. The prognostic factors depend on the quality of the surgical excision, the presence of metastases and certain locations (head, neck), which make the surgery particularly mutilating. Only long-term monitoring attests to definitive cure, given the frequency of recurrence. CONCLUSION: DFSC is a rare and slowly evolving tumour. Wide surgical excision should be attempted in most cases. In inoperable cases, the use of targeted therapies (IMATINIB) has led to complete cures in some cases.

Comparing Mohs micrographic surgery and wide local excision in the management of head and neck dermatofibrosarcoma protuberans: a scoping review.

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 Occurring in an Adult Presenting with Chronic Lymphedema: A Case Study and Literature Review.

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue tumor typically located in the trunk. We report a unique case of DFSP in the right inguinal region of an adult presenting with chronic lymphedema. Only 1 case of DFSP and chronic lymphedema association has been previously reported in the literature. Since we could not provide adjuvant radiotherapy (RT), we conducted an extensive review of its application in similar cases, exploring various surgical treatments. CASE REPORT A 42-year-old Cameroonian man with unexplained chronic lymphedema presented with a tumor in the inguinal region of the affected limb. The patient underwent wide local excision (WLE) of the mass, including regional lymph node dissection. Pathological exam confirmed DFSP with a fibrosarcomatous component. Adjuvant RT was considered but not pursued due to the patient;s non-compliance. CONCLUSIONS This DFSP is reported for its rarity of site and the unique co-occurrence with chronic lymphedema. Considering both conditions are uncommon and the rarity of site of the DFSP, we assume that in this patient, chronic lymphedema was a contributing factor of occurrence of the DFSP. Remarkably, no prior reports have detailed an association between chronic lymphedema and DFSP onset. For that reason, we want to point out the value of better follow-up of chronic lymphedema and better knowledge of DFSP treatment options to improve patient healthcare and limit DFSP recurrence. In addition, we found adjuvant RT is an interesting treatment option that might be considered in all patients undergoing surgical excision, even in cases where negative surgical margins were achieved.

Effective resection of dermatofibrosarcoma protuberans using the novel Saturn's rings technique.

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade sarcoma with locally infiltrative characteristics. A wide excision is made with a 5 cm gross margin. However, recently, Mohs micrographic surgery has emerged as an alternative. In this study, we evaluate a novel method (Saturn's Rings technique) for removing tumors that combines the advantages of wide excision and Mohs micrographic surgery. Between September 2001 and March 2020, 29 patients with DFSP, excluding the head and neck, were treated using Saturn's rings technique. Frozen biopsies were performed at regular intervals under various conditions and depths. If the biopsy was positive, a negative margin was obtained through an additional session. The depth and breadth of excision, reconstruction method, and postoperative recurrence were analyzed. The breadth of excision was evaluated at 1 cm intervals. Only 1 session was required in 12 cases, 2 sessions were needed in 13 cases, and 3 or more sessions were performed in 4 cases. In 24 cases, the depth of excision was limited to above the superficial fascia, with a negative biopsy of the superficial fascia. Local flaps were the most common reconstruction method (n = 21, 72.4%). No cases of recurrence were observed at follow-up (average, 45.7 months). Saturn's rings technique for DFSP maximized the preservation of normal tissue with minimal resection, was advantageous for reconstruction, and yielded good results without recurrence.

Incidence and Survival Outcomes of Dermatofibrosarcoma Protuberans From 2000 to 2020: A Population-Based Retrospective Cohort Analysis.

BACKGROUND: Recent changes in the incidence and survival of dermatofibrosarcoma protuberans (DFSP) have not been described. OBJECTIVE: To characterize the incidence and survival of DFSP. MATERIALS AND METHODS: A retrospective cohort study of patients with DFSP from 2000 to 2020 in the Surveillance, Epidemiology, and End Results database was performed. Cox and Fine-Gray regression models were used to assess overall and DFSP-specific survival. RESULTS: The incidence of DFSP has not changed from 2000 to 2020 with 4.6 cases/million person-years, with higher rates in dark-skinned and middle-age individuals. Factors associated with overall mortality in DFSP patients include advanced age ( p < .0001), male sex (hazard ratio [HR] 1.8, p < .0001), larger tumors (HR 1.002 per millimeter, p < .001), lower household income (HR 1.8, p = .0002), and lower extremity location (HR 1.7, p = .008). Mohs surgery is associated with improved overall survival (HR 0.4, p = .02). Large tumor size (6.0+ cm, HR 6.7, p = .01) and advanced age (age 80+ years, HR 21.3, p = .003) were associated with worse DFSP-specific mortality. CONCLUSION: Dermatofibrosarcoma protuberans incidence has remained constant from 2000 to 2020. Increasing age and tumor size, decreased income, male sex, and lower extremity location are associated with worsened survival. Mohs surgery is associated with improved overall survival. Increased age and tumor size are associated with worsened DFSP-specific mortality.