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.

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.

[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.

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.

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.

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.

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.

Pigmented dermatofibrosarcoma (Bednar tumour) of scalp.

Pigmented dermatofibrosarcoma protuberans (DFSP) also known as Bednar tumour is a very rare variant of DFSP which is considered to be of intermediate grade along with the presence of melanin-containing dendritic cells. Only a handful of cases have been described in the literature so far. It is centred around the dermis or subcutis and can pose a diagnostic challenge by being confused with other pigmented lesions of the skin. We hereby report one such unusual case of a man in his late 20s presenting with swelling over the forehead for the past 7 years. Hence a diagnosis of pigmented DFSP should always be considered while reporting pigmented subcutaneous lesions with spindle cell morphology.

Dermatofibrosarcoma protuberans: A rare presentation in the scalp. Case report.

BACKGROUND: Dermatofibrosarcoma protuberans of the scalp (DFSP) is a rare soft tissue neoplasm originating from the dermal layer of the skin, usually affecting the adults. CASE REPORT: The current case report presents a 48-year old male with a huge lump on the right side of parietal region. A wide local excision of the tumor was performed and the excised specimen was sent for histopathological examination. Histopathology and Immunohistochemistry was suggestive of DFSP. CONCLUSION: Dermatofibrosarcoma protuberans is a rare neoplasm affecting the head and neck region. This unusual entity is more likely to recur when a small margin of surgical excision is performed. Wide local excision is the gold standard treatment and radiotherapy is preferred in recurrent diseases.

Survival Outcomes and Prognostic Factors of Dermatofibrosarcoma Protuberans: A Population-Based Retrospective Cohort Analysis.

BACKGROUND: Limited information is available regarding survival outcomes and risk factors of dermatofibrosarcoma protuberans (DFSP). OBJECTIVE: To investigate the clinicopathologic characteristics and survival outcomes of DFSP. MATERIALS AND METHODS: The study cohort (7,567 patients) was selected from the Surveillance, Epidemiology, and End Results Program (2000-2018). Demographic and clinicopathologic variables, survival outcomes, and prognostic factors were analyzed. RESULTS: A total of 5,640 (74.53%) and 1,927 (25.47%) tumors were located in the skin and soft tissue, respectively. The median follow-up duration was 92 months. Median follow-up times were similar between patients with lymph node (107 months) and distant (102 months) metastases; the median survival time of the 89 patients (1.18%) who died of DFSP was significantly short (41 months, p < .001). Independent risk factors for cancer-specific mortality included age at diagnosis, histologic grade, and tumor size. Patients with tumors ≥10 cm in size or histologic grade III had significantly higher DFSP-specific mortality (7.07% and 10.08%, respectively, p < .001). Tumor locations and surgical procedures did not significantly influence survival. CONCLUSION: Dermatofibrosarcoma protuberans has a favorable survival prognosis, even in patients with node-positive or distant metastases. Dermatofibrosarcoma protuberans-specific mortality is significantly higher in patients with grade III or large (≥10 cm) tumors.

Recurrent dermatofibrosarcoma protuberans: A report of two cases involving the trunk and abdominal wall.

In this paper, we report two cases of dermatofibrosarcoma protuberans (DFSP) who were treated with distinct surgical approaches. In the first case, a 50-year-old woman presented with a mass on her right shoulder and underwent local excision with subsequent reconstruction using a deltopectoral flap. The second case was of a young female who presented with a giant protuberant DFSP on the anterior abdominal wall who was treated with wide local excision along with inlay mesh repair of the defect. Early excision and adjuvant radiotherapy facilitates a low recurrence rate while also improving the prognosis of the patients.

Machine learning-based radiomics analysis for predicting local recurrence of primary dermatofibrosarcoma protuberans after surgical treatment.

BACKGROUND AND PURPOSE: Dermatofibrosarcoma protuberans (DFSP) is characterized by locally invasive growth patterns and high local recurrence rates. Accurately identifying patients with high local recurrence risk may benefit patients during follow-up and has potential value for making treatment decisions. This study aimed to investigate whether machine learning-based radiomics models could accurately predict the local recurrence of primary DFSP after surgical treatment. MATERIALS AND METHODS: This retrospective study included a total of 146 patients with DFSP who underwent MRI scans between 2010 and 2016 from two different institutions: institution 1 (n = 104) for the training set and institution 2 (n = 42) for the external test set. Three radiomics random survival forest (RSF) models were developed using MRI images. Additionally, the performance of the Ki67 index was compared with the three RSF models in the external validation set. RESULTS: The average concordance index (C-index) scores of the RSF models based on fat-saturation T2W (FS-T2W) images, fat-saturation T1W with gadolinium contrast (FS-T1W + C) images, and both FS-T2W and FS-T1W + C images from 10-fold cross-validation in the training set were 0.855 (95% CI: 0.629, 1.00), 0.873 (95% CI: 0.711, 1.00), and 0.875 (95% CI: 0.688, 1.00), respectively. In the external validation set, the C-indexes of the three trained RSF models were higher than that of the Ki67 index (0.838, 0.754, and 0.866 vs. 0.601, respectively). CONCLUSION: Random survival forest models developed using radiomics features derived from MRI images were proven helpful for accurate prediction of local recurrence of primary DFSP after surgical treatment and showed better predicting performance than the Ki67 index.