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PRAME (PReferentially expressed Antigen in MElanoma) was first identified as a malignant melanoma-specific antigen. Recently, a few cases of fibrosarcomatous dermatofibrosarcoma protuberans (FS-DFSP) were shown to have positivity for PRAME, while conventional dermatofibrosarcoma protuberans (C-DFSP) was negative. Because PRAME may be of diagnostic utility in FS-DFSP and is raising expectations as a new immunotherapy target, we examined the positivity of PRAME in FS-DFSP. Twenty-one cases of FS-DFSP and age/sex/location-matched cases of C-DFSP as a control group were examined by immunohistochemistry for CD34 and PRAME. The results were then evaluated by H-score, which was objectively and semi-quantitatively calculated using the open-source bioimaging analysis software QuPath. The results revealed that the PRAME H-score in FS-DFSP was significantly higher than that in C-DFSP ( p  = 0.0137). As for CD34, the H-score in FS-DFSP was significantly lower than that in C-DFSP ( p  < 0.001). Using these two immunohistochemical analyses in combination, the sensitivity and specificity for the diagnosis of FS-DFSP were 86% and 90%, respectively. Double staining of CD34 and PRAME revealed that PRAME-positive and CD34-positive areas did not overlap. This is the largest study to examine PRAME expression in FS-DFSP, and it confirmed the usefulness of PRAME in diagnosing this condition.

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.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor with a high incidence of misdiagnosis. DFSP has a high rate of recurrence but a low rate of metastasis. In the present study, retrospective analyses were performed on the clinical features, differential diagnosis and treatment of patients with DFSP to improve our understanding of the disease and allow more effective treatment measures to be implemented. The present study investigated the clinicopathological features of 70 pathologically confirmed cases of DFSP at the First Affiliated Hospital of Zhengzhou University (Zhengzhou, China) between March 2012 and 2017. The primary endpoint was recurrence rate. Three cases were analyzed in detail. The results revealed that 7 of the 41 primary patients (follow-up at 2.7 years) had recurrence (17.1%), compared with 11 (37.9%) of the 29 recurrent patients (follow-up at 2.0 years, P=0.049). Of the 59 patients with DFSP (follow-up at 2.6 years), 12 had recurrence (20.3%) compared with 6 (54.6%) of the 11 patients with fibrosarcomatous DFSP (FS-DFSP; follow-up at 2.1 years, P=0.045). DFSP requires diagnosis by pathological examination, and surgical resection is the main treatment. DFSP demonstrated a high recurrence rate, with the degree of malignancy increasing following multiple recurrences. FS-DFSP had a higher risk of local recurrence and distant metastasis, and a higher degree of malignancy than classic DFSP. These data may be useful to guide clinicians to improve decisions in the treatment of patients with DFSP.

Background Autologous adipose tissue transfer may be performed for aesthetic needs following the resection of dermatofibrosarcoma protuberans (DFSP), the most common cutaneous soft tissue sarcoma, excluding Kaposi sarcoma. The regenerative effectiveness of cell-assisted lipotransfer is dependent on the presence of adipose tissue-derived stem cells (ADSCs). This is the first study to evaluate the potential oncological risks as ADSCs could unintentionally be sited within the proximity of the tumor microenvironment of DFSP cells. Methods Primary DFSP cells were indirectly co-cultured with ADSCs in a conditioned medium or in a Transwell system. The impact was analyzed by assessing proliferation, migration, invasion, angiogenesis, and tumor-associated genes and proteins. Results of these assays were compared between co-culture and mono-culture conditions. Results Our experimental results showed that ADSCs were able to promote proliferation, migration, invasion, and angiogenesis of DFSP cells; this was accompanied by a significant increase in the expression levels of beta-type platelet-derived growth factor receptor, collagen type I alpha 1 chain, vascular endothelial growth factor, hepatocyte growth factor, and basic fibroblast growth factor. Conclusions The current report clearly demonstrates that ADSCs can enhance different malignant properties of DFSP cells in vitro, which should not be neglected when considering the clinical use of human ADSCs and its related derivatives in skin regenerative therapies.

Background Dermatofibrosarcoma protuberans (DFSP) may recur locally but rarely metastasizes. Fibrosarcomatous transformation in dermatofibrosarcoma protuberans (FS-DFSP) is said to have worse prognosis compared with ordinary DFSP (O-DFSP). Since DFSP rarely metastasizes, there have been few reports summarizing data on distant metastasis cases at single institution. The aim of this retrospective study is to review DFSP cases in order to analyze risk factors for metastasis. Patients and methods This retrospective study involved 67 patients. We analyzed O-DFSP and FS-DFSP metastasis rates, metastasis sites, time to metastasis, the relationship between frequency of local recurrence and metastasis, and the relationship between primary tumor size and metastasis. Results Distant metastasis was found in 5 (7.4 %) of 67 cases with DFSP. Of the five cases, the histopathological diagnosis was FS-DFSP in four cases and O-DFSP in one case. Out of five cases with metastasis, three had not recurred and two had recurred twice. No clear correlation was identified (Fisher’s exact test: p  = 0.216). The primary tumor diameters in the metastatic cases were 15.0, 12.6, 20.5, 13.0, and 5.0 cm, respectively. The tumor diameters in metastatic cases were significantly larger (Fisher’s exact test: p  < 0.0001). Conclusions In this study, we identified a stronger correlation between DFSP metastasis and tumor size. There was a high possibility that the cases with large tumors might be FS-DFSP, having high rate of metastasis and poor prognosis. In treatment of DFSP, early diagnosis before primary tumor growth and wide resection is considered important. Level of evidence V.

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation (DFSP‐FS) is a rare variant, with higher rates of recurrence and metastasis than DFSP. Detection of the collagen type I alpha 1 (COL1A1)–platelet‐derived growth factor beta chain (PDGFB) fusion gene is useful for the diagnosis of DFSP. In this letter, we report a case of DFSP‐FS, focusing on the expression of the COL1A1‐PDGFB fusion gene in the lesions. Increased expression of the COL1A1‐PDGFB fusion gene may be associated with fibrosarcomatous changes during the pathogenesis of DFSP. Increased expression of the COL1A1‐PDGFB fusion gene may be associated with fibrosarcomatous changes during the pathogenesis of DFSP.

Dermatofibrosarcoma protuberans（DFSP）, the most common dermal sarcoma, is a low-grade, slow growing fibroblastic malignant neoplasm that most frequently affects middle aged adults and is characterized by a high local recurrence rate and a low propensity for metastasis. Wide surgical resection or Mohs micrographic surgery（MMS） are the preferred approaches for localized disease, while radiation therapy is warranted for inoperable disease or for cases with positive margins where re-excision is not possible. DFSP is generally regarded as refractory to conventional chemotherapy. Treatment options for systemic disease were limited until the discovery of a unique translocation, t（17;22）（q22;q13）（COL1A1;PDGFB） found in a majority of cases. In recent years, imatinib, a PDGFβR, ABL and KIT inhibitor, has revolutionized systemic therapy in DFSP. In this review, we summarize the epidemiological, clinical, histological and genetic characteristics of DFSP and update the readers on its current management.

Dermatofibrosarcoma Protuberans (DFSP) is a rare soft tissue sarcoma distinguished by its infiltrative growth pattern and recurrence potential. Understanding the molecular characteristics of DFSP is essential for enhancing its diagnosis, prognosis, and treatment strategies. The paper provides an overview of DFSP, highlighting the significance of its molecular understanding. The gene expression profiling has uncovered unique molecular signatures in DFSP, highlighting its heterogeneity and potential therapeutic targets. The Platelet-Derived Growth Factor Receptors (PDGFRs) and Fibroblast Growth Factor Receptors (FGFRs) signaling pathways play essential roles in the progression and development of DFSP. The abnormal activation of these pathways presents opportunities for therapeutic interventions. Several emerging therapies, i.e., immunotherapies, immunomodulatory strategies, and immune checkpoint inhibitors, offer promising alternatives to surgical resection. In DFSP management, combination strategies, including rational combination therapies, aim to exploit the synergistic effects and overcome resistance. The article consisting future perspectives and challenges includes the discovery of prognostic and predictive biomarkers to improve risk stratification and treatment selection. Preclinical models, such as Patient-derived xenografts (PDX) and genetically engineered mouse models, help study the biology of DFSP and evaluate therapeutic interventions. The manuscript also covers small-molecule inhibitors, clinical trials, immune checkpoint inhibitors for DFSP treatment, combination therapies, rational therapies, and resistance mechanisms, which are unique and not broadly covered in recent pieces of literature.