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Aims: The majority of dermatofibrosarcoma protuberans (DFSP) harbour PDGFB or PDGFD rearrangements. We encountered ALK expression/rearrangement in a PDGFB/D‐negative CD34‐positive spindle cell neoplasm with features similar to DFSP, prompting evaluation of ALK‐rearrangements in DFSP and plaque‐like CD34‐positive dermal fibroma (P‐LDF). Methods and Results: We searched the archives of academic institutions for cases previously coded as DFSP and P‐LDF. NGS‐naïve or PDGFB‐negative DFSP were screened for ALK (clone D5F3) expression by immunohistochemistry. NGS or ALK FISH was performed on ALK‐positive cases. Methylome profiling studies were performed and compared with conventional DFSP. One case of "DFSP" and two "P‐LDF" with ALK expression were identified from the archives, while four cases were detected prospectively. These seven cases (6F:1M; 8 months to 76 years) arose in the dermis of the arm (two), scalp, eyelid, thigh, abdomen, and shoulder and ranged from 0.4 to 4.2 cm. Tumours were composed of spindled cells and displayed a storiform growth pattern. Cytologic atypia was absent, and mitotic figures were scarce (0–2/10 HPFs, high power fields). The lesional cells were diffusely positive for CD34 and ALK and negative for S100 protein. By NGS (n = 5), ALK fusion partners included DCTN1 (2), PLEKHH2, and CLIP2 in DFSP‐like cases and FLNA in P‐LDF‐like lesions. ALK FISH was positive in one (of two) cases previously labelled P‐LDF. Methylome profiling of two (of three) ALK‐rearranged DFSP‐like tumours showed clustering with conventional DFSP in the UMAP dimension reduction plot. To date, no tumour has recurred (n = 2; 26, 27 months). Conclusion: We describe a cohort of novel ALK‐rearranged tumours with morphologic features similar to DFSP. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma characterized by an asymmetric, infiltrative growth pattern and a high risk of local recurrence. This study aims to evaluate the effectiveness of various imaging modalities in the assessment and management of DFSP. Nine imaging modalities were reviewed including: Ultrasound (US), High‐Frequency Doppler Ultrasound (HFUS), Computed tomography (CT), Positron emission tomography–computed tomography (PET‐CT), and Magnetic Resonance Imaging (MRI), High‐resolution‐MRI (HR‐MRI), Magnetic Resonance Spectroscopy (MRS), Optical Coherence Tomography (OCT), and Dermatoscopy. Imaging is mainly used for preoperative assessment and surgical planning, not routine diagnosis. US is effective for initial evaluations, demonstrating superior ability in detecting muscle invasion and defining tumour boundaries (sensitivity – 81.8%, specificity – 100%). MRI is valuable for preoperative evaluation, surgical planning, and monitoring DFSP recurrence. It more accurately assesses tumour depth than palpation, with a sensitivity of 67% and specificity of 100%, but was inferior when compared to US. CT is utilized in cases of suspected bone involvement or pulmonary metastasis. For advanced or recurrent DFSP, PET‐CT helps manage treatment responses and imatinib therapy. Emerging technologies like MRS and OCT show potential in improving diagnostic accuracy and defining surgical margins, though more data are needed. US, MRI, and CT are the primary imaging modalities for DFSP. Emerging technologies like HR‐MRI, PET‐CT, MRS, and OCT hold promise for refining diagnostic and management strategies. Integrating multiple technologies could enhance management, particularly in atypical or aggressive cases. Further studies are required to refine imaging protocols and improve DFSP outcomes. [ABSTRACT FROM AUTHOR]

Aims: The majority of dermatofibrosarcoma protuberans (DFSP) harbour PDGFB or PDGFD rearrangements. We encountered ALK expression/rearrangement in a PDGFB/D‐negative CD34‐positive spindle cell neoplasm with features similar to DFSP, prompting evaluation of ALK‐rearrangements in DFSP and plaque‐like CD34‐positive dermal fibroma (P‐LDF). Methods and Results: We searched the archives of academic institutions for cases previously coded as DFSP and P‐LDF. NGS‐naïve or PDGFB‐negative DFSP were screened for ALK (clone D5F3) expression by immunohistochemistry. NGS or ALK FISH was performed on ALK‐positive cases. Methylome profiling studies were performed and compared with conventional DFSP. One case of "DFSP" and two "P‐LDF" with ALK expression were identified from the archives, while four cases were detected prospectively. These seven cases (6F:1M; 8 months to 76 years) arose in the dermis of the arm (two), scalp, eyelid, thigh, abdomen, and shoulder and ranged from 0.4 to 4.2 cm. Tumours were composed of spindled cells and displayed a storiform growth pattern. Cytologic atypia was absent, and mitotic figures were scarce (0–2/10 HPFs, high power fields). The lesional cells were diffusely positive for CD34 and ALK and negative for S100 protein. By NGS (n = 5), ALK fusion partners included DCTN1 (2), PLEKHH2, and CLIP2 in DFSP‐like cases and FLNA in P‐LDF‐like lesions. ALK FISH was positive in one (of two) cases previously labelled P‐LDF. Methylome profiling of two (of three) ALK‐rearranged DFSP‐like tumours showed clustering with conventional DFSP in the UMAP dimension reduction plot. To date, no tumour has recurred (n = 2; 26, 27 months). Conclusion: We describe a cohort of novel ALK‐rearranged tumours with morphologic features similar to DFSP. [ABSTRACT FROM AUTHOR]

Simple Summary: Dermatofibrosarcoma protuberans (DFSP) is a rare proliferative condition representing skin sarcomas which is known to locally recur yet very rarely metastasizes. Its genetic background is a reciprocal translocation t(17;22)(q22;q13) that produces COL1A1-PDGFB gene fusion. Complete resection is the primary treatment. The aim of this reappraisal is to review various correlates of the condition. Our review underlines that cases of low-to-moderate grade DFSP with excellent prognosis must be distinguished from high grade fibrosarcomatous DFSP with a much worse prognosis. Dermatofibrosarcoma protuberans (DFSP) is a rare proliferative condition representing skin sarcomas which is known to locally recur yet very rarely metastasizes. Its genetic background is a reciprocal translocation t(17;22)(q22;q13) that produces COL1A1-PDGFB gene fusion. Complete resection is the primary treatment. The aim of this review is to outline the pathogenesis, diagnosis, and management of DFSP. A clear-cut distinction between low-to-moderate-grade DFSP with excellent prognosis and high-grade fibrosarcomatous DFSP with a much worse prognosis is underlined. Malignant transformation within DFSP (or high histologic grade), older age, being female, large primary tumor size (≥10 cm), narrow surgical margins of excision (<3 cm), surgical margin positivity for tumor cells, short time to recurrence, numerous recurrences, tumor that was recently rapidly enlarging, and presence of pain in the tumor have all been proposed as clinicopathological risk factors for recurrence and metastasis. A tendency for local growth and local relapses of well- and moderately differentiated DFSPs is an argument for their surgical excision, possibly combined with reconstructive surgery, even in patients of advanced age. Another main point of this review is that cases of DFSP with fibrosarcomatous transformation are a challenge and require careful medical attention. Both anatomopathological evaluation of the presence of lymphovascular space invasion and sentinel lymph node biopsy at DFSP surgery merit further study. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Fibroblastic connective tissue nevus (FCTN) is a rare, benign dermal mesenchymal lesion of fibroblastic and myofibroblastic lineage. We report a case of a 2‐year‐old male who presented with an 18‐month history of an erythematous, asymptomatic, unchanging dermal plaque on the right medial frontal scalp. A punch biopsy showed a disorderly, bland, dermal fibroblastic spindle cell proliferation extending to the superficial subcutis. It stained positive for CD34, and concern for dermatofibrosarcoma protuberans was raised. However, FISH was negative for PDGFB rearrangement, and the constellation of findings was most consistent with FCTN. This case underscores the importance of distinguishing CD34+ mesenchymal tumors for both dermatologists and dermatopathologists. As these represent a rather diverse group of lesions with different biological behaviors, a knowledge of the differential diagnosis of these entities is critical for proper patient management. [ABSTRACT FROM AUTHOR]

Background Dermatofibrosarcoma protuberans (DFSP) is one of the most challenging cutaneous cancers in surgical clinic practice. Excision with negative margins is essential for effective disease control. However, wide surgical margins and maximal tissue conservation are mutually exclusive. Mohs micrographic surgery conserves tissue but is time-consuming. Thus, we developed a novel specimen radiography system that can be used intraoperatively. Aims To introduce a specimen radiography system for evaluating intraoperative surgical margins in patients with dermatofibrosarcoma protuberans. Methods Since September 2017, we have treated seven biopsy-proven cases of local DFSPs via local excision with surgical margins of 2–4 cm. During operations, the operative specimens were screened using the specimen radiography system. All surgical specimens were pathologically examined intraoperatively. Results Five patients were men and two were women, of median age 36 years. The mean radiographic screening time was 9.7 ± 2.3 min. Radiographically negative margins were confirmed intraoperatively. The minimal margin width ranged from 5.0 to 35.4 mm (mean width 16.9 ± 10.4 mm). The intraoperatively negative radiographic margins were consistent with those revealed by postoperative pathology. The minimal pathological margin width ranged from 4.0 to 34.5 mm (mean 16.6 ± 10.1 mm) and was not significantly different from the intraoperative data. Limitations The sample size was small and positive or negative predictive values were not calculated. Conclusions We introduce a novel method of intraoperative surgical margin assessment for DFSP patients. It may find broad clinical and research applications during oncoplastic surgery. [ABSTRACT FROM AUTHOR]

Simple Summary: While sun exposure and associated tissue changes stemming from ultraviolet radiation are closely associated with the most common forms of skin cancer, far less is known regarding rare types of skin cancer. In this study, for the first time, we used a light microscopy technique to evaluate connective tissue changes in samples from patients with six different types of rare skin cancers, assessing the relationship between these changes, patient age, and whether tumors arose on sun-exposed parts of the body. We found that these tissue changes were most pronounced for patients with specific cancers known to be linked to chronic sun damage and tumors arising on sun-exposed parts of the body. We also noted tumor type-specific trends in terms of sex ratios, sites of tumor presentation, and the relationship between the development of particular tumors and patient immunosuppression. Our results are important and novel as they expand the available data associated with these rare skin cancers while also offering insight into the value of differentiating among these tumor types based on their relationship with sun exposure, potentially informing preventative, diagnostic, and/or therapeutic approaches. (1) Background: Rare skin cancers include epithelial, neuroendocrine, and hematopoietic neoplasias as well as cutaneous sarcomas. Ultraviolet (UV) radiation and sunburns are important drivers for the incidence of certain cutaneous sarcomas; however, the pathogenetic role of UV light is less clear in rare skin cancers compared to keratinocyte cancer and melanoma. In this study, we compared the degree of actinic elastosis (AE) as a surrogate for lifetime UV exposure among selected rare skin cancers (atypical fibroxanthoma [AFX], pleomorphic dermal sarcoma [PDS], dermatofibrosarcoma protuberans [DFSP], Kaposi sarcoma [KS], Merkel cell carcinoma [MCC], and leiomyosarcoma [LMS]) while taking into account relevant clinical variables (age, sex, and body site). (2) Methods: We newly established a semi-quantitative score for the degree of AE ranging from 0 = none to 3 = total loss of elastic fibers (basophilic degeneration) and multiplied it by the perilesional vertical extent (depth), measured histometrically (tumor-associated elastosis grade (TEG)). We matched the TEG of n = 210 rare skin cancers from 210 patients with their clinical variables. (3) Results: TEG values were correlated with age and whether tumors arose on UV-exposed body sites. TEG values were significantly higher in AFX and PDS cases compared to all other analyzed rare skin cancer types. As expected, TEG values were low in DFSP and KS, while MCC cases exhibited intermediate TEG values. (4) Conclusions: High cumulative UV exposure is more strongly associated with AFX/PDS and MCC than with other rare skin cancers. These important results expand the available data associated with rare skin cancers while also offering insight into the value of differentiating among these tumor types based on their relationship with sun exposure, potentially informing preventative, diagnostic and/or therapeutic approaches. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a cutaneous sarcoma with a high propensity for local invasion and recurrence. Although it is a rare event, the occurrence of multiple tumors in a single patient raises a diagnostic dilemma, as metastatic disease should be differentiated from multiple primary malignant events. In more than 90% of DFSP, a pathogenic t(17;22) translocation leads to the expression of COL1A1::PDGFB fusion transcripts. Karyotype analysis, fluorescence in situ hybridization, and RT‐PCR can be useful ancillary studies in detecting this characteristic rearrangement, and sequencing of the fusion transcript can be used to support a clonal origin in metastatic and multifocal disease. However, previous reports have demonstrated variable sensitivity of these assays, in part due to the high sequence variability of the COL1A1::PDGFB fusion. Here, we report a patient who developed two distinct DFSP tumors over the course of 7 years. Chromosomal microarray analysis identified distinctive genomic alterations in the two tumors, supporting the occurrence of multiple primary malignant events. [ABSTRACT FROM AUTHOR]

Background: Diffractive microscopy creates contrast within samples that are otherwise uniform under bright light. This technique can highlight subtle differences in refractive indices within birefringent samples containing varying amounts of mature collagen. Dermatofibroma (DF) and dermatofibrosarcoma protuberans (DFSP) possess differences in their mature collagen content and, therefore, may be distinguishable using diffractive microscopy. Methods: Two hundred forty‐two DF and 85 DFSP hematoxylin–eosin (H&E)‐stained specimens were analyzed using diffractive microscopy. Data regarding the distribution pattern and strength of refractility was recorded. Results: DFSP was more frequently found to be focally, weakly, or non‐refractile (82.9%; n = 68) under diffractive microscopy, while DF more often showed diffusely bright refractility (52.9%; n = 128). DFSP samples with diffuse refractility in portions of the lesion (17.1%; n = 14) also exhibited a unique checkerboard pattern distinct from that which was seen in DF samples. Conclusions: The absence of diffuse refractility was more closely associated with DFSP, as was the presence of a unique checkerboard diffraction pattern. Despite high sensitivity (Sn = 82.9%), absent refractility was not a specific test (Sp = 52.9%), with 47.1% (n = 114) of DF samples sharing this feature. The distinction between DF and DFSP is often diagnosed using H&E alone. In difficult cases, examination of collagen under diffractive microscopy may be useful in distinguishing DFSP from DF and provide an alternative cost‐effective tool to immunohistochemical staining. [ABSTRACT FROM AUTHOR]

Background: Head and neck dermatofibrosarcoma protuberans (HNDFSP) is extremely rare and not entirely understood. Objective: To investigate the clinicopathological features of HNDFSP and identify the expression of its clinically relevant indicators, with the expectation of improving the existing treatment strategies. Methods: A long‐term follow‐up of patients with HNDFSP who received treatment between 2000 and 2021 at Shanghai Ninth People's Hospital was conducted. The clinical, histological, and immunohistochemical data of the patients were retrieved and analyzed. The endpoint of the study was the incidence of significant disease‐related clinical events (recurrences or metastasis). Results: A total of 49 patients with HNDFSP were included in the study, with males (92.7%) predominating than females (7.3%). Eighteen patients developed recurrent disease (36.8%) after surgery, and the median time of recurrence was 48 months (interquartile, 20–74 months). Metastasis occurred in two cases (4.1%). Two patients died during follow‐up, both with local recurrence, and one of them with intestinal metastasis. Post‐operation radiotherapy was administered to eight patients (16.3%) and the effect in local control was remarkable. Age, tumor size, and negative margins with sufficient safety width were the main independent factors affecting the disease‐free survival. Several potential targeted therapeutic indicators, including EZH2 (80.0%), EGFR (91.4%), PDGF (97.1%), PD‐L1 (77.1%), and VEGF (77.1%), were positively expressed in most tumor samples. Conclusion: HNDFSP is rare, significantly challenging to control locally, and has a worse prognosis with current treatment strategies. Wide local excision and long‐term follow‐up are needed. Radiotherapy could improve the prognosis of patients with HNDFSP. [ABSTRACT FROM AUTHOR]

Background: Dermatofibrosarcoma protuberans (DFSP) is a superficial soft tissue sarcoma, and surgical excision is the first-line treatment. The aim of this systematic review is to provide an update about the current indications and clinical results regarding the use of postoperative radiotherapy in DSFP, considering both adjuvant and salvage setting. Methods: We conducted a systematic literature review using the main scientific database, including Cochrane library, Scopus, and PubMed, for any relevant article about the topic, and we considered all available papers without any time restriction. Results: Twenty-two papers, published between 1989 and 2023, were retrieved and considered eligible for inclusion in this review. Regarding the fractionation schedules, most authors reported using standard fractionation (2 Gy/die) with a wide total dose ranging from 50 to 70 Gy. The local control after postoperative radiotherapy was excellent (75–100%), with a median follow-up time of 69 months. Conclusions: After the primary surgical management of DFSP, postoperative radiotherapy may either be considered as adjuvant treatment (presence of risk factors, i.e., close margins, recurrent tumours, aggressive histological subtypes) or as salvage treatment (positive margins) and should be assessed within the frame of multidisciplinary evaluation. [ABSTRACT FROM AUTHOR]

Cutaneous fibrous histiocytoma (FH) is considered a benign dermal tumor. The cellular variant is rare and poorly documented. Besides presenting a high risk of local recurrence, it has a low but serious metastatic potential. We present a case of metastatic cellular FH and also review the literature on this tumor, given its unusual metastatic development. A 47‐year‐old male patient presented with a lesion in the anterior surface of the right thigh, which has been present since adolescence but had grown during last year. Anatomopathological evaluation revealed a cellular FH, and the lesion was completely removed. Six months later, tumor recurrence with multiple compartment muscle involvement and pulmonary metastasis were detected. Both lesions were completely resected and after 3 years of follow‐up, the patient is asymptomatic and free of the disease. We conclude that FH should be carefully sampled to detect variants with high local recurrence rates or with some metastatic risk such as the cellular one. We recommend wide surgical resection and a close follow‐up including chest x‐rays or thorax computed tomography (CT) in all cellular FH cases with local recurrence. [ABSTRACT FROM AUTHOR]

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. Samples from patients who underwent surgery between 2010 and 2018 were collected. Intralesional and peripheral (at 2 cm 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 2 cm margins. Secondly a FISH analysis was used as a quantitative diagnostic approach, in order to find the t(17;22) translocation. Among the seven specimens that included 2 cm margins, two samples presented one or more translocations, which were not visible in standard morphology assessments at two centimeters tumor-free margins. 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. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a low-grade malignant soft-tissue tumor that originates from the skin. It has a slow onset in the early stages, non-specific clinical symptoms, low specificity, and can easily be overlooked, missed, or misdiagnosed by clinicians and pathologists. In addition, DFSP is prone to recurrence after local surgical treatment; however, distant metastasis, especially abdominal metastasis, is rare, which is also a challenge for the accurate diagnosis of DFSP when it progresses distantly. Now a case of abdominal metastasis of DFSP is reported. The patient has been treated with imatinib for ten years, and the lesion has shrunk, but because the patient has been receiving imatinib treatment, his abdominal lesion was once misdiagnosed as gastrointestinal stromal tumor. Therefore, we report on this case to enhance the understanding of the diagnosis and treatment of DFSP, and to provide reference for the pathological diagnosis and precise treatment of such patients. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is an infrequent cutaneous tumour that may involve subcutaneous fat and in some cases fascia, muscles and bone. The infrequent occurrence lessens its clinical awareness in addition to its clinical semblance to many common cutaneous lesions. It is characterized by proclivity for local recurrence. We evaluated the clinical presentation and treatment outcomes of six consecutive cases of DFSP with histologic diagnosis in the University of Calabar Teaching Hospital, Calabar seen between January 2013 and December 2022. This was compared with total cutaneous malignances in the same period. The six consecutive patients comprised of two males and four females (M = F: 1:2) whose ages ranged from 21 to 57 years (mean of 36.5 years) and accounted for 7% of cutaneous malignancies. The site distribution was trunk (back) involved in 3 (50%) of the patients, limbs 3 (50%); upper 1 (17%) and lower limb 2 (33%). Clinical presentation was in the form of firm cutaneous mass with some ulcerated lesions that bled and some fungated. 50 percent of the patients presented with recurrent lesions and in all there was no regional lymphadenopathy or evidence of metastasis. There was a patient with Neurofibromatosis- 1 who had a huge fungated limb lesion offered amputation, four had wide local excision and one incision biopsy. Follow up was poor and the period ranged from 2 to 14 months (mean 7 months). Dermatofibrosarcoma protuberans is an uncommon tumour with clinical semblance to other cutaneous lesions. Early presentation, preoperative histologic diagnosis will enhance the goal of ensuring adequate excision. Adjuvant therapy with Imatinib with or without adjuvant radiotherapy are recommended in the treatment plan in view of the frequency of late presentation with advanced recurrent lesions and poor follow up. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans is a rare low-grade sarcoma, which rarely metastasizes, but it is locally aggressive with a propensity to recur. It usually affects persons of African descent and is extremely rare in childhood with a favourable prognosis. We present a case of paediatric dermatofibrosarcoma protuberans to the midline of the lower back of a 9-year-old Afro-Caribbean boy who was biopsied with a 2-mm margin. After histological confirmation, a 4-cm margin was then performed. Surveillance for recurrence, though none has been seen thus far after 6-month follow-up, will be done for at least 5 years and possibly longer, given this is the first case of this nature ever seen in our institution and the Caribbean region. [ABSTRACT FROM AUTHOR]

Background: Pilomatricoma has various manifestations on color Doppler ultrasound, and a differential diagnosis is challenging. The objective of this study was to investigate which characteristics of skin lesions on color Doppler ultrasound are effective in distinguishing pilomatricoma from epidermoid cyst and dermatofibrosarcoma protuberans. Materials and methods: Records of patients with pilomatricomas (n = 63), epidermoid cysts (n = 76), and dermatofibrosarcoma protuberans (n = 19) who underwent color Doppler ultrasound evaluation and surgical excision were reviewed. The anatomical distribution and color Doppler ultrasound characteristics of these lesions were analyzed. The 63 pilomatricomas were categorized into five types based on their color Doppler ultrasound characteristics, and the roles of these five types in the differential diagnosis of the aforementioned diseases were studied. Results: Pilomatricomas, epidermoid cysts, and dermatofibrosarcoma protuberans exhibited some similar characteristics. Dominantly markedly hyperechoic or hyperechoic appearance, posterior acoustic shadowing, and the presence of vascularity were the major characteristics of pilomatricomas. The pilomatricomas could be categorized into five types, with type II having a diagnostic performance of sensitivity of 65.08%, specificity of 98.95%, area under the receiver operating characteristic curve (AUC) of 0.743, positive predictive value of 97.62%, and negative predictive value of 81.03% for the diagnosis of the aforementioned skin diseases. Conclusion: A combination of dominantly markedly hyperechoic or hyperechoic appearance, posterior acoustic shadowing, and the presence of vascularity exhibits higher diagnostic performance for the differential diagnosis of pilomatricomas, epidermoid cysts, and dermatofibrosarcoma protuberans. [ABSTRACT FROM AUTHOR]

Simple Summary: Rare diseases represent a major health problem, since patients face difficulties in obtaining a rapid diagnosis and appropriate treatments. Vulvar dermatofibrosarcoma protuberans is one of these rare entities, in which reaching a correct pathological diagnosis is intricate and surgical techniques are not standardised. The aim of our paper is to review the available literature on vulvar dermatofibrosarcoma protuberans to summarise previous experiences and main issues, in an attempt to improve the management of this rare disease. Dermatofibrosarcoma protuberans of the vulva needs to be diagnosed early and managed by a referral centre, where the patient can receive appropriate management: surgical treatment should aim to obtain free margins, lowering the probability of recurrence. Long-term follow up is needed, since recurrences are documented even after several years. Background: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding appropriate disease management. Methods: We made a systematic search of the literature, including the terms "dermatofibrosarcoma protuberans", "vulva", and "vulvar", alone or in combination. We selected articles published in English from two electronic databases, PubMed and MEDLINE, and we analysed their reference lists to include other potentially relevant studies. Results: We selected 39 articles, with a total of 68 cases reported; they were retrospective case reports and case series. Dermatofibrosarcoma protuberans of the vulva tends towards local recurrence; an early and timely pathological diagnosis, together with an appropriate surgical approach, are of utmost importance to ensure free margins and maximise the curative potential. Conclusions: Even if this is an indolent disease and it generally shows a good prognosis, appropriate management may help in reducing the rate of local recurrences that may hamper patients' quality of life. Management by a multidisciplinary team is highly recommended. [ABSTRACT FROM AUTHOR]

Introduction: Dermatofibrosarcoma protuberans (DFSP) originated as keloid sarcoma, gaining its current designation in 1925. DFSP exhibits slow growth, categorizing it as a low- to intermediate-grade malignant sarcoma. Initially presenting as a small, firm, irregular skin nodule, it undergoes sudden, rapid growth, forming a prominent mass. While locally aggressive, distant metastasis is rare. DFSP affects mainly the torso then proximal extremities. Case Presentation: In this case study, we described a 57-year-old male individual who presented with a chest midline swelling that was progressing in size. A punch biopsy showed inconclusive results. Thus, a wide local excision was carried out along with sending the initial biopsy slides to Mayo Clinic for second opinion. A diagnosis of DFSP was confirmed, which is an uncommon and locally aggressive tumor affecting soft tissues. The primary histological diagnosis relies on immunohistochemical stains, enabling the distinction between DFSP and other fibrous tumors. Conclusion: Diagnosing DFSP is challenging due to its similarity to other skin lesions. A multidisciplinary approach is vital for accurate diagnosis and management. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare form of skin cancer characterized by local aggressiveness, typically affecting individuals in the young to middle-aged adult range. The authors of this article aimed to present the clinical picture, as well as the therapeutic approach to a patient undergoing oncological surveillance with DFSP, analyzing the therapeutic response rate based on data published in specialized journals. A 72-year-old man was admitted to the radiation oncology department, having a 75/58 mm tumoral mass on the right posterior cervical region. The patient underwent two radical tumor excision surgeries and interval skin grafting, leading to a negative prognosis for the patient, as the tumor reappeared aggressively in a short period, four months, more exactly. The study highlighted the importance of surgical intervention, such as wide local excision, in preventing recurrence and the major role of initiating timely adjuvant treatment, including radiotherapy. The previously performed studies suggest that radiotherapy can be beneficial following resection with negative margins, and it can serve as primary therapy for patients with inoperable or incompletely excised DFSP. In this case, the only therapeutic response was stopping the progression of the tumor growth and macroscopic changes of the tumor surface with areas of necrosis. This study also discusses the use of imatinib as the only left therapeutic option, due to the lack of radiotherapy response. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) stands as a rare and locally aggressive soft tissue tumor, characterized by intricated molecular alterations. The imperative to unravel the complexities of intratumor heterogeneity underscores effective clinical management. Herein, we harnessed single-cell RNA sequencing (scRNA-seq) to conduct a comprehensive analysis encompassing samples from primary sites, satellite foci, and lymph node metastases. Rigorous preprocessing of raw scRNA-seq data ensued, and employing t-distributed stochastic neighbor embedding (tSNE) analysis, we unveiled seven major cell populations and fifteen distinct subpopulations. Malignant cell subpopulations were delineated using infercnv for copy number variation calculations. Functional and metabolic variations of diverse malignant cell populations across samples were deciphered utilizing GSVA and the scMetabolism R packages. Additionally, the exploration of differentiation trajectories within diverse fibroblast subpopulations was orchestrated through pseudotime trajectory analyses employing CytoTRACE and Monocle2, and further bolstered by GO analyses to elucidate the functional disparities across distinct differentiation states. In parallel, we segmented the cellular components of the immune microenvironment and verified the presence of SPP1+ macrophage, which constituted the major constituent in lymph node metastases. Remarkably, the CellChat facilitated a comprehensive intercellular communication analysis. This study culminates in an all-encompassing single-cell transcriptome atlas, propounding novel insights into the multifaceted nature of intratumor heterogeneity and fundamental molecular mechanisms propelling metastatic DFSP. [ABSTRACT FROM AUTHOR]

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing, and locally aggressive soft tissue tumor with a high recurrence rate and metastatic potential, even with the proper treatment.Methods: This was a retrospective (case series) study that took place at King Abdulaziz Medical City, Riyadh, Kingdom of Saudi Arabia, to determine the outcomes of and appropriate margin excision for DFSP. All patients who were diagnosed with DFSP from 2016 to 2021 were included. The following variables were assessed: demographics, tumor characteristics, management options, and most importantly, whether patients were managed with an oncology-oriented approach or a non-oncology-oriented approach.Results: There were a total of seventeen patients with DFSP, four of whom had fibrosarcomatous differentiation (FS-DFSP). The majority (N = 13, 76.5%) of the patients were females. The lower extremities and back were the most common locations for DFSP, accounting for 47.1% and 23.5%, respectively. Only two (11.76%) patients had metastatic disease, one of whom had FS-DFSP. The minimum resection margin was 3 cm, and the maximum was 5 cm. Thirteen (76.47%) patients were managed with an oncology-oriented approach (Group I), 23% of whom had post-excision positive margins. However, all patients who were managed with a non-oncology-oriented approach (Group II) had positive margins post-excision. More than three-quarters (76.9%) of group I underwent wide resection. Split-thickness skin grafting and primary closure were the most commonly used reconstruction methods in groups I and II, respectively. The mean planned margins in groups I and II were 3.9 cm and 1.7 cm, respectively.Conclusion: The findings of this study suggest that a planned wide-margin excision with a minimum safe margin of 3– 5 cm should be implemented to reduce the recurrence, metastasis, and need for further surgeries in patients with DFSP. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma Protuberans (DFSP) is a rare, locally aggressive fibroblastic mesenchymal neoplasm, typically derived from the dermis, with the intramammary subtype being seen infrequently. We present a case of a 40-year-old woman whom was diagnosed with an intramammary DFSP during pregnancy, whom underwent successful surgical management during her second trimester. Our case demonstrates the importance of increased clinical awareness in the diagnosis and treatment of breast DFSP with careful multidisciplinary consideration. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is rare, generally slow growing tumor, originating from dermal stem cell or undifferentiated mesenchymal cell. DFSP primarily occurs on the trunk and proximal extremities, with reported only 10–15% occurring in the head and neck region. There can be varied presentations of DFSP in atypical sites mimicking a benign appearance and history due to which diagnostic dilemmas can occur. The underlying malignancy needs to be unveiled by histopathology and immunohistochemistry which reveals bland spindle cells in sub epithelium arranged in whorled storiform pattern which was CD34+, suggestive of dermatofibrosarcoma protuberans. Surgical excision is the treatment of choice, wherever possible. We herein report two intriguing cases of DFSP in uncommon sites. [ABSTRACT FROM AUTHOR]

We encountered a giant dermatofibrosarcoma protuberans (DFSP) of the neck and chest wall which presented a challenge in terms of perioperative analgesia management. In recent years, erector spinae plane (ESP) block has emerged as an efective and safe analgesia technique for various surgical procedures as well as for chronic neuropathic pain without any untoward complications. A continuous lower cervical ESP block can be used successfully as an efective analgesic technique for extensive DFSP surgery involving the neck and chest wall area. [ABSTRACT FROM AUTHOR]

Under the umbrella of cutaneous sarcomas (CS) we include a heterogeneous group of rare, malignant, mesenchymal neoplasia, such as dermatofibrosarcoma protuberans, atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, cutaneous angiosarcoma and leiomyosarcoma. Clinical presentation and histopathological examination are the cornerstone of CS diagnosis and classification. There are scarce data in the literature in regards to the clinical and dermatoscopic characteristics of CS and the role of dermatoscopy in their early identification. We performed a literature review, aiming to summarize current data on the clinical and dermatoscopic presentation of the most common types of cutaneous sarcomas that may facilitate early diagnosis and prompt management. Based on the available published data, CS are characterized by mostly unspecific dermatoscopic patterns. Dermatofibrosarcoma protuberans, Kaposi's sarcoma, and in a lesser degree, cutaneous angiosarcoma, may display distinct dermatoscopic features, facilitating their early clinical recognition. In conclusion, dermatoscopy, in conjunction with the overall clinical context, may aid towards suspicion of CS. [ABSTRACT FROM AUTHOR]

Background: Dermatofibrosarcoma protuberans (DFSP) of the breast is a dermal fibroblastic neoplasm requiring wide excisional margins due to recurrence rates ranging from 26 to 60%. The current literature on reconstructive options and utility of Mohs micrographic surgery for DFSP of the breast is scarce. We describe surgical management of DFSP of the breast at our institution with the largest case series reported to date. Methods: A retrospective review was performed of women who underwent surgery for DFSP of the breast at our institution between 1990 and 2019. Continuous data was summarized using mean, median, and range; categorical data was summarized with frequency count and percentage. Preoperative lesion size and postoperative defect size were evaluated using 2-sided Fisher exact test, and p-values < 0.05 were considered statistically significant. Results: Nine patients underwent wide local excision (WLE) with reconstruction including pedicled latissimus dorsi flaps (n = 2), local flap advancement (n = 2), mastectomy with implant (n = 1), oncoplastic breast reduction (n = 1), and skin grafts (n = 3). Nine underwent Mohs micrographic surgery (MMS) with complex primary closure. Mean postoperative maximum wound defect size for WLE was 10.8 cm versus 7.0 cm for MMS with no statistical significance (p = 0.77). Mean preoperative maximum lesion size for WLE was 6.4 cm versus 3.3 cm for MMS with no statistical significance (p = 0.07). Complications with WLE included wound dehiscence in three patients and seroma in one patient. No complications were reported with MMS and primary closure. Recurrence was reported in one WLE patient, which was successfully detected despite flap coverage and resected without complications. Median follow-up for the patients without recurrence was 5.0 years, with two patients in MMS cohort lost to follow-up. Five-year overall survival was 100%. Conclusions: MMS and WLE are both viable surgical options for managing DFSP of the breast. MMS could potentially minimize reconstructive needs due to smaller average defect size and result in fewer complications but may also result in asymmetry. Immediate flap reconstruction, especially in larger defects, can achieve excellent aesthetic outcomes for patients with DFSP of the breast without compromising detection of disease recurrence. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a locally aggressive soft tissue tumor with a high propensity of local recurrence after surgery. Radiotherapy as an adjuvant therapy has been shown to reduce recurrent rates of this disease. Surface mould brachytherapy is an effective and safe modality for the delivery of radiotherapy in soft tissue tumors, though its utilization and popularity have decreased in recent years. Here, we presented a case of a recurrent DFSP of the scalp who was treated with surgery followed by adjuvant surface mould brachytherapy to avoid dose inhomogeneity likely to occur in this anatomic region with external beam radiotherapy in the absence of intensity-modulated radiotherapy. The treatment was delivered successfully with minimal adverse reactions, and the patient is disease-free at 18 months post-treatment with no treatment toxicity. [ABSTRACT FROM AUTHOR]

Background: There is limited data on how demographics, tumor characteristics, and treatment methods affect overall survival in patients with dermatofibrosarcoma protuberans (DFSP). Objective: To summarize characteristics of patients with DFSP, assess prognostic factors, and evaluate the impact of treatment modality on their overall survival. Methods: We investigated DFSP using data for 4451 patients with histologically confirmed cases of DFSP diagnosed between 2000 and 2018 from the 18 US regional registries of the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) Program. Results: Older age (P <.001) and large tumor size (P =.006) were significantly associated with worse overall survival in controlled analysis. Older age (P <.050), males (P <.001), non-white race (P <.001), and lower median household income (P <.010) were more likely to present with larger tumor size. Different treatments were associated with patient characteristics. Older age (P <.001), non-white race (P <.032), larger tumor size (P <.001), and head/neck location (P <.001) were associated with patients receiving surgery and radiation instead of surgery only. Additionally, men (P <.021), non-whites (P <.001), lower median household income (P <.001), and larger tumor size (P =.003) were less likely to have Mohs micrographic surgery performed over excision. Conclusions: Age at presentation and tumor size appeared to be notable prognostic factors. Although treatment modality did not significantly influence patient survival, certain patient characteristics are associated with different treatment modalities. [ABSTRACT FROM AUTHOR]

BACKGROUND: Erosive pustular dermatosis is a neutrophilic dermatosis of chronic course, characterized by the appearance of pustules, erosions and scabs. Although it typically affects the scalp, it can appear in any cutaneous territory. The diagnostic-therapeutic management of this disease is a real challenge when it affects special areas. CLINICAL CASE: A 45-year-old male patient went under Mohs micrographic surgery for a dermatofibrosarcoma protuberans and he presented a surgical wound on right shoulder with an unfavourable healing. After months of treatment with a split-thickness skin graft, punch grafting and negative wound pressure therapy, the diagnosis of erosive pustular dermatosis secondary to negative wound pressure therapy and the practice of skin grafts was reached. A spectacular relieve of the wound was observed, and complete epithelialization was achieved in two weeks after the withdrawal of negative wound pressure therapy and the application of topical corticosteroids. CONCLUSIONS: We report to our knowledge the first case of erosive pustular dermatosis secondary to negative wound pressure therapy and skin graft. Being aware of erosive pustular dermatosis when treating a post-surgical wound may help to promptly detect this dermatological condition and might help to carry out an early alternative therapy to avoid more aggressive procedures. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing malignancy that often presents with an ambiguous clinical presentation due to its nonspecific findings. We present the case of a 41-year-old male who presented with a slow-growing mass on his abdomen that became protuberant and firm, prompting his desire to pursue excision. Upon return of histologic examination, the specimen was noted to be DFSP. DFSP is a rare cutaneous neoplasm that originates from the dermis and invades the underlying tissue creating a classic protuberant appearance. In some cases, chemotherapy and radiation may be indicated based on margins and locations. The sarcoma is notoriously complicated with early recurrence, making the disease process difficult to control. Surgeons should be familiar with this malignancy due to the indication of additional nonsurgical treatments and the necessity for long-term follow-up for surveillance of recurrence. [ABSTRACT FROM AUTHOR]

Sarcomas represent 1% of malignancies in adult population; thereby dermatofibrosarcoma protuberans is found in 1%–2% of all cases. A surgical approach in oncologic treatment is the standard of care; therefore, important is an extensive resection to achieve clear margins and prevent recurrence. Herein we report a case of recurrent dermatofibrosarcoma protuberans in the thoracolumbar region. As the adequate resection was to cause a huge defect, we have made a reconstruction plan for coverage, using a perforator-based double Keystone Island flap (Type III). The method was chosen as a reliant solution with low complication rates and without need for complex flap designs thereby giving good functional and esthetic results. Main aim of the case report was to show that Keystone Island flaps present a simple and technically straightforward method with low complication rates and good results. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction: Dermatofibrosarcoma protuberans (DFSP) is an uncommon skin tumor with a low-to-intermediate grade of malignancy, characterized by progressive growth and a propensity for local recurrence. In this series, we are analyzing the clinicopathological spectrum of DFSP cases. Materials and Methods: A retrospective study of 12 patients with DFSP who were diagnosed at our institute over the last 2 years (2018–2020) was performed. Results: The clinicopathological spectrum and immunohistochemistry of DFSP cases were studied with one case with lymph node metastasis, which is a rare entity, and two cases of the fibrosarcomatous DFSP were also diagnosed and warrant a special mention. Conclusion: Pathologists should be aware of metastasis and unusual variants while reporting dermatofibrosarcoma. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive spindle cell mesenchymal tumor arising in the dermis, with low metastatic potential. The most commonly affected sites are the trunk and proximal extremities; rarely are acral sites involved. Atrophic DFSP is a rare form of DFSP, that is morphologically different but histologically similar to DFSP. It commonly affects young adults between the ages of 20 to 50 years. The current management strategy for atrophic DFSP is surgical excision with long-term follow-up to detect any recurrence. Only one known case of atrophic DFSP with eosinophilic infiltration is what makes our case an exceptionally rare presentation. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans is a very rare tumour that accounts for less than 0.1% of all malignant neoplasms. It is a locally aggressive tumour with high recurrence rate. Head and neck involvement is very uncommon and hence diverse treatment protocols have been recommended for its management in the currently available literature. We present a case of dermatofibrosarcoma protuberans over the lower part of right cheek in a middle aged male, which was successfully treated with wide local excision and reconstruction along with post-op radiotherapy. The patient was followed-up for 18 months with no recurrence. Prompt and accurate diagnosis along with multidisciplinary treatment is crucial for optimal management of this rare tumor. The optimal treatment option is surgical resection with wide margins along with adjuvant radiotherapy, which leads to better outcomes even in patients with positive surgical margins. Larger clinical trials with Imatinib will firmly establish its chemotherapeutic role in its management. Lifelong regular follow-up is essential for early detection of tumor. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma of the dermis. It is a malignant, locally aggressive, and infiltrative tumor with frequent recurrence. In this case, a 44-year-old woman presented with a 15-year history of a swelling in the medial canthus of the right eye that caused tearing. Imaging revealed a septated mass isodense to soft tissue that had eroded the medial wall of the orbit. Macroscopic examination showed an elastic, gray-brown, encapsulated, irregular mass measuring 45x35x22 mm. The surgical margins were positive, so adjuvant radiotherapy was started. The patient was followed for 2 years without recurrence. According to the literature, this mass is the largest orbital DFSP treated by globe-sparing primary resection. [ABSTRACT FROM AUTHOR]

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare, low to intermediate-grade sarcoma, which needs imaging examination. Small series of ultrasound findings in DFSP have been published; however, the usefulness of elastography and contrast-enhanced ultrasound (CEUS) in DFSP has not been studied. We aim to study multimodal ultrasound findings and report the correlation between imagings and tiny extension in DFSP for preoperative evaluation.Methods: Two-D ultrasound, 3-D color ultrasound, elastography, and CEUS findings were retrospectively evaluated. Forty histopathologically confirmed DFSPs were studied.Results: On 2-D ultrasound, 26(65%) appeared as mostly hypoechoic lesions with occasional hyperechoic dots within the tumor matrix and lobulated lateral borders. Eight (20%) lesions were multilayered. Ninety-five percent of lesions showed increased vascularity. On 3-D ultrasound, DFSPs showed branch-shaped, striped, and wrapped color patterns. Power Doppler showed mainly artery of a moderate arterial peak systolic blood flow and low resistance index. DFSP is hard on elastography. On CEUS, DFSPs showed a long peak time, low peak and a small amount of perfusion around the tumor, 73.7% (14/19) of lesions showed a heterogeneous contrast enhancement and 89.5% (17/19) of lesions showed hyper-enhancement. CEUS showed better concordance than US with histology on the maximum diameter and depth (P < 0.05).Conclusions: Multimodal ultrasound showed significant characteristics in DFSP, which would improve diagnostic accuracy. CEUS could be an effective tool to determine tiny tumor extension. [ABSTRACT FROM AUTHOR]

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare fibrohistiocytic tumour of unknown pathogenesis with intermediate malignant potential. Although trauma has been hypothesized as a predisposing factor for DFSP development, clinicopathological characteristics of trauma‐related DFSP have not been investigated. Objective: This study investigated the differences between trauma‐associated DFSP and trauma‐unrelated DFSP. Methods: Patients histopathologically diagnosed with DFSP from January 2000 to December 2019 at the Dermatology Department were included. Clinical, histopathological, prognostic features and trauma history were analysed. Results: We recruited 141 patients with DFSP (mean age, 36.1 years; male: female, 1:1.01). Recurrence and systemic metastasis were observed in 15.6% and 2.8% of patients, respectively. Older patients were likely to develop DFSP at the trauma sites more frequently on the face and lower legs. The active‐growing lesions were more frequently associated with trauma‐related DFSP. Multivariable logistic regression revealed that age at diagnosis (OR: 1.031; 95% CI: 1.004–1.059; p = 0.024) and tumour growth (OR: 3.336; 95% CI: 1.162–9.578, p = 0.025) were significantly associated with trauma‐related DFSPs. Conclusions: The age at diagnosis, lesion location and tumour growth were associated with DFSPs in this study. Analysis of DFSP with trauma history provides a deeper understanding of long‐term trauma effects on sarcoma development. [ABSTRACT FROM AUTHOR]

Head and neck dermatofibrosarcoma protuberans (HNDFSP) is a rare neoplasm with a high rate of local infiltration and local recurrence but a low rate of distant metastasis. Given the limited literature on HNDFSP and existing studies pointing to possible racial and ethnic differences, further research is needed to understand important clinical correlates that may impact treatment and prognosis. The National Cancer Database (NCDB) was queried for all cases of HNDFSP diagnosed from 2004 to 2016. Demographic characteristics of the included patients were compared using chi-squared and t-tests. Kaplan-Meier and Cox multivariable regression analyses were performed to assess survival differences. Of the 778 included patients, 526 (67.6%) patients were white, 113 (14.5%) were black, 33 (4.2%) were Asian, 87 (11.2%) were Hispanic, and 19 (2.4%) were classified as Other. White patients presented at a significantly older age (43.8 years) than did black (38.9 years) and Hispanic patients (37.9 years) (p = 0.02). White patients with HNDFSP had significantly higher income status (p = 0.0001), were more likely to be insured (p = 0.0001), and were more likely to have a high school diploma (p = 0.0001). There were no significant racial differences in 5-year (96.1%) or 10-year (92.0%) overall survival. None of the variables yielded a statistically significant value in Cox analysis. Differences exist in HNDFSP presentation between racial groups. Specifically, differences in income, insurance status, education level, and age of presentation exist between these groups. Future studies focused on morbidity are needed to better understand the consequences of these difference on HNDFSP patients. [ABSTRACT FROM AUTHOR]

Dermatofibrosarcoma protuberans [DFSP] is a low to intermediate soft tissue sarcoma arising from the skin dermis. DFSP of the scalp is very rare and is often confused with an epidermoid cyst. Patient Concern: Women reported with a swelling on the scalp for 6 months. Rational: The purpose of the report is to highlight the variable presentations of DFSP. Report: We report a case of DFSP of the scalp in a middle-aged woman. Diagnosis: The lesion histologically showed spindle-shaped cells and was positive for S100 and Ki 67 and negative for CD34. Outcome: The lesion was excised and the margins were negative for tumor cells. The patient was followed up for 2 months and there were no recurrences reported. Lessons: One must be aware of this uncommon entity and always perform a wide excision for these tumors to reduce the risk of recurrence. [ABSTRACT FROM AUTHOR]

Background: Dermatofibrosarcoma protuberans (DFSP), an uncommon sarcoma of the dermis, is a low-grade, spindle-cell tumor that usually grows slowly and has a high local recurrence rate, but rarely metastasizes to distant sites. The diagnosis is based on histopathology and the standard treatment is wide surgical excision with tumor-free margins. Case Report: Described is the case of a 75-year-old female who presented with a giant tumor on her upper right arm that had been increasing in size for a year. A biopsy provided the diagnosis of DFSP. Radical surgery was performed and the arm was disarticulated from the scapula. Conclusion: Treatment for DFSP typically involves surgical excision; however, the size of the tumor in the present case was exceptionally large. Disarticulation offered the best prognosis. [ABSTRACT FROM AUTHOR]

Objective: Racial and ethnic health disparities affect the diagnosis and management of melanoma and nonmelanoma skin cancers, leading to deleterious outcomes. Non-Hispanic White patients make up the majority of skin cancers cases, yet racial and ethnic minorities have poorer prognoses and outcomes. The skin cancer literature is fragmented with regards to potential contributors to these healthcare disparities. In this article, we provide a comprehensive review of the skin cancer literature to briefly quantify racial and ethnic inequities, highlight contributing factors, and propose practical changes that can be made.Methods: A PubMed search was completed to identify articles related to racial and ethnic health care disparities in the context of melanoma, basal cell carcinoma, squamous cell carcinoma, Merkel cell carcinoma, and dermatofibrosarcoma protuberans.Results: Relative to non-Hispanic White patients, patients of racial and ethnic minorities have differing clinical presentations of skin cancers and genetic risk factors. Insurance, access to specialty care, cultural beliefs, and available educational resources further contribute to racial and ethnic disparities.Limitations: We are limited to the level of detail provided in the existing literature, and at some times are unable to distinguish race of Hispanic populations. We also acknowledge that there are different nationalities grouped under these broad labels as well as multi-racial populations that may not be accounted for.Conclusion: Awareness of and familiarization with innate factors and potentially more modifiable contributors can help inform efforts to close the observed gap in racial and ethnic inequities. [ABSTRACT FROM AUTHOR]

BACKGROUND: Dermatofibrosarcoma protuberans is a low to intermediate grade skin sarcoma that affects dermis and subcutaneous tissue. CLINICAL CASE: A 30-year-old female patient with no significant personal history who consulted our department for a 9-year-old tumor lesion, located in the right labia majora, which recurred after conventional surgical excision. By means of a pathology and immunohistochemical study, the diagnosis of vulvar dermatofibrosarcoma protuberans was confirmed, an infrequent entity in a location little reported in the literature. CONCLUSIONS: Dermatofibrosarcoma protuberans is a low-frequency sarcoma-type skin cancer that involves dermis and subcutaneous cellular tissue. It usually affects people in the third to fourth decade of life, and it is more common in the extremities and trunk. It is a slow-growing, low-recurrence tumor that rarely spreads beyond the skin. The vulvar location is rare, occurring in less than 60 cases reported to date in the literature. The definitive diagnosis is made with the histopathological study and within the therapeutic options there are surgical alternatives and systemic therapies. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To evaluate the level of correlation between the clinical diagnosis of dermatofibrosarcoma protuberans and the results of histopathology and immunohistochemistry. MATERIALS AND METHODS: Observational, cross-sectional, correlational, retrospective and retrolective study was made including all clinical records of dermatofibrosarcoma protuberans from the Dermatopathology Unit of the Hospital General de México Dr. Eduardo Liceaga, Mexico City, from January 1st, 1976 to December 31st, 2019. RESULTS: Forty-six cases of dermatofibrosarcoma protuberans were analyzed, 29 were women. It represented the second most frequent cutaneous sarcoma, the most frequent histological subtype was the classic one and the storiform pattern predominated; furthermore, immunohistochemical markers for CD34 were positive in 18/46 cases and Ki67 in 11/46. The most frequent clinical diagnosis prior to taking the biopsy was dermatofibrosarcoma protuberans. CONCLUSIONS: The classic dermatofibrosarcoma protuberans is the most frequent in our environment, with a storiform pattern and honeycomb-shaped infiltration of the fat. There was a statistically significant difference among various contrasted variables, such as mitoses and age, clinical diagnosis and topography, in terms of histology, the histological subtype and age, the histological pattern and subtype, the cell type and histological subtype. [ABSTRACT FROM AUTHOR]

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. 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. [ABSTRACT FROM AUTHOR]

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade soft tissue sarcoma of dermal origin having a high local recurrence rate. It usually affects adults between the age group of second and fifth decade. It usually involves the trunk, proximal extremities, head, and neck regions rather than at the vulva region. It is clinically significant being having a high local recurrence rate. The study aims to find out and discuss on the literature review regarding this tumour occurring in a rare location. Method: We presented a case of DFSP from a 54-year-old married woman with an exophytic mass at the vulvar region. Multiple sectioning including its surgical margins were thoroughly performed. Histopathological examination with conventional H&E stain as well as immunohistochemical (IHC) procedure using CD 34 stain was applied for the confirmation and differentiation from other possible spindle cell tumours. Result: We were able to identify a highly cellular lesion running in sheets and parallel bundles. At a first glance it appeared like a spindle cell lesion originated from either smooth muscles or fibrous tissue. Hence, we proceeded with CD34 IHC stain to confirm and exclude other possible differentials. A strong positivity to CD 34 IHC of the lesion was observed. A thorough pathologic assessment with multiple sectioning is an absolute necessity in reporting DFSP occurring in an uncommon region. It is also necessary to express the clearance of its surgical margins to help avoid potential recurrence of the lesion. Conclusion: The role of immunohistochemistry is an essential procedure in confirmation of DFSP from other possible spindle cell tumours: both benign and malignant lesions [ABSTRACT FROM AUTHOR]

Aims: The study highlights diffusion-weighted imaging (DWI) and dynamic enhancement features of DFSP and characterizes unenhanced and enhanced computed tomography (CT) and magnetic resonance imaging (MRI) scans.Settings and Design: Image findings and clinical histories of 23 patients with DFSP were reviewed. Nine patients underwent CT before and after intravenous administration of contrast material. MRI was performed for 17 patients. CT and MRI findings were analyzed using location, size, edge, shape, infiltration sign, density and signal enhancement mode, and degree.Results: Patients showed 26 superficial and one deep lesion. Ten superficial lesions bulged onto the skin surface. Fourteen lesions were well-defined and 13 ill-defined. All lesions were nodular, with nine being multilobular. Thirteen showed infiltration to adjacent skin, fat, and fascia. Seven lesions on CT were iso- or hypo-dense to muscle without calcification. Contrast-enhanced CT showed inhomogeneous moderate and progressive enhancement in the arterial phase. Small tortuous vessels were seen in the arterial phase in one case. Sixteen tumors displayed signals that were similar to muscle by T1WI. Ten lesions were either hyper-intense to muscle or iso-intense to fat; the deep DFSP was hypo-intense by T2WI. All lesions were hyper-intense homogeneously or heterogeneously under fat-suppressed T2WI. Twelve superficial lesions showed high-intermediate signal, and one deep lesion showed low-intermediate signal with DWI. Seven cases showed low signal diffusion coefficient (ADC) images. Dynamic enhancement and signal intensity-time (SI-T) curves of four tumors showed rapid SI increases followed by steady or slightly rising SI. All lesions showed inhomogeneous, progressive enhancement in the arterial phase.Conclusions: This report is the first on dynamic curves and highlights DWI and T2WI features of DFSP. DFSP can be correctly diagnosed by combining a patient's clinical manifestations with imaging characteristics. [ABSTRACT FROM AUTHOR]