Dermatofibrosarcoma Research Articles

LETTER TO THE EDITOR: THORACO-ACROMIAL ARTERY PERFORATOR (TAAP) FLAP FOR RECONSTRUCTION OF A RECURRENT DERMATOFIBROSARCOMA PROTUBERANS OF THE CLAVICULAR REGION

Dermatofibrosarcoma Protuberans is a rare mesenchymal tumor, highly infiltrative and with a high recurrence rate. Despite its localization may vary, the trunk is widely recognized as the most common interested site. We read with interest the recent article published by Longo et al. “Recur-rent dermatofibrosarcoma protuberans of the clavicular region: radical excision and reconstruction with Latissimus Dorsi myocutaneous flap”; since their case is very similar to one we faced in 2016. In this letter, we present our experience with the Thoraco-acromial artery perforator flap (TAAP) for reconstruction of a recurrent DFSP of the clavicular region.

Fine needle aspiration cytology of dermatofibrosarcoma protuberans: A rare case report

Introduction: Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous spindle cell tumor with incidence 0.8 to 5 cases/million population/year.1 found almost exclusively in the dermis, from which they often invade the subcutaneous tissue. It is a slow-growing, locally aggressive tumor of intermediate malignancy. It poses a diagnostic challenge when encountered on fine needle aspiration cytology (FNAC) owing to its rarity and characteristics that are indistinguishable from other spindle cell lesions. It has high tendency for local recurrence but low rate of distant metastasis.Case report: 26 years old female presented in department of surgery as a mass in the epigastric region which was painless and progressive since 6 months. Ultrasonography was done which was suggestive of neoplastic etiology with possibility of dermatofibrosarcoma protuberans. Patient was advised FNAC.Methods: FNAC was done without local anaesthesia and smears were made. Smears were fixed with methanol and stained by PAP’s stain, H&E stain and unfixed slides were stained by MGG stain. Detailed cytomorphological features were studied.Result: Smears showed spindle cell tumor suggestive of DFSP on cytology and it was confirmed by histopathology.Conclusion: Distinguishing dermatofibrosarcoma protuberans from other spindle cell tumors and fibrohistiocytic lesions may pose significant challenges to the pathologist. However, in the appropriate clinical setting and applying strict diagnostic criteria, fine needle aspiration cytology is a reliable tool in establishing the diagnosis of this neoplasm.

Giant exophytic dermatofibrosarcoma protuberans of the breast

Dermatofibrosarcoma protuberans (DFSP) is a rare neoplasm characterized by a high rate of local recurrence and a low risk of metastases. Occurrence in the breast is extremely rare and requires an adequate preoperative diagnosis for a correct management. We report a case of a giant exophytic DFSP of the right breast in a young-aged woman. Radical mastectomy was carried out without clinical evidence of local recurrence after 2 years follow-up. Specific pathological features and recommendations for the clinical management are discussed.

Reconstruction options in recurrent dermatofibrosarcoma protuberans:A scoping review.

This review will outline the evaluation, diagnosis, and management of dermatofibrosarcoma protuberans and emphasizes multidisciplinary role of nurses, plastic surgeons and radiation oncologist in this recurrent metastatic lesion. It pinpoints affected population at risk, clinical features, and reconstruction options. No analytical research has been done in this area. A scoping review of patients of DFSP who underwent reconstruction after excision of tumors was performed in the Department of Burn and Plastic Surgery, AIIMS Rishikesh. It used a five framework approach. A review of 85 similar cases reported in the literature have been scrutinized in relation to the reconstruction options, sites of the tumor, margins of excision and recurrence. 85 full length English studies were included out of the 445 cases found in Pubmed and related search engines to reveal various reconstructive options in reconstruction of DFSP defects. Present scoping review identifies free anterolateral thigh flap to be useful in 7 review articles followed by propeller flaps in 3 isolated case reports. 2 cases of free latissimus dorsi flap were used for reconstruction of abdominal defects. All patients should undergo a strict screening protocol where the health personnel can play a crucial role by educating parents on the follow up and report new lesions as early as possible. All operated tumor patients can be given safety tips and education on care and risks after reconstruction with skin flaps or skin grafting. A multidisciplinary approach between the surgeon, nurse and radiation oncologist is needed for effective management of these lesions.

Dermatofibrosarcoma protuberans of the upper extremity: a case report

Dermatofibrosarcoma protuberans of the upper extremity: a case report

Clinical and histopathological analysis of 141 dermatofibrosarcoma protuberans in Korea: A comparative study according to trauma.

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. This study investigated the differences between trauma-associated DFSP and trauma-unrelated DFSP. 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. 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. 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.

Comprehensive analysis of transcriptome characteristics and identification of TLK2 as a potential biomarker in dermatofibrosarcoma protuberans.

Background: Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma characterized by local invasion and recurrence. RNA sequencing (RNA-seq) allows the qualification of cellular RNA populations and provides information on the transcriptional state. However, few studies have comprehensively analyzed DFSP transcriptional data. Methods: Fourteen DFSP samples with paired non-neoplastic soft tissue from Chinese patients undergoing Mohs micrographic surgery were used for RNA-seq analysis. Differential expression analysis and enrichment analysis for RNA-seq data were performed to identify fusion genes, biomarkers, and microenvironment characteristics of DFSP. Results: This study systemically describes the transcriptomic characteristics of DFSP. First, we performed gene fusion analysis and identified a novel FBN1-CSAD fusion event in a DFSP patient with fibrosarcomatous transformation. Then, we identified TLK2 as a biomarker for DFSP based on functional enrichment analysis, and validated its accuracy for diagnosing DFSP by immunohistochemical staining and joint analysis with public data. Finally, microenvironment analysis described the infiltration characteristics of immune and stromal cells in DFSP. Conclusion: This study demonstrates that RNA-seq can serve as a promising strategy for exploring molecular mechanisms in DFSP. Our results provide new insights into accurate diagnosis and therapeutic targets of DFSP.

Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery is associated with lower odds of postoperative radiotherapy compared to wide local excision

Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery is associated with lower odds of postoperative radiotherapy compared to wide local excision

Recurrent Dermatofibrosarcoma Protuberans of the Head and Neck: a Case Series.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma that develops from dermal fibroblasts and spreads within the dermis and subcutaneous fat. It is locally aggressive, with a high local recurrence rate after excision but has extremely low metastatic potential. In the case of recurrent tumors, surgical excision with adequate margins is the gold standard treatment and may require adjuvant radiotherapy or chemotherapy in some cases. We conducted a retrospective analysis of individuals with dermatofibrosarcoma protuberance of the head and neck region that had treatment at our facility between 2016 and 2021. We gathered the data on the surgical techniques, reconstructive techniques used, histopathological features, adjuvant therapy, and outcomes. We treated three patients with head and neck dermatofibrosarcoma protuberance: one scalp lesion and two on the cheek. All three patients had recurrent tumors, two of whom were treated elsewhere for the primary lesion. One patient underwent surgery for a benign spindle cell tumor of the right cheek, but a final histopathological examination revealed dermatofibrosarcoma protuberance, and the tumor recurred within 3months. The duration of recurrence is between 3 and 24months. The size of the tumor ranges from 7.2 to 10.5cm. The wide local excision margins range from 2 to 4cm. Reconstruction ranges from split skin graft to regional flap. Inadequate margins raise the possibility of local recurrence in dermatofibrosarcoma protuberance.

35224 Identifying a correlation between preceding trauma and development of dermatofibrosarcoma protuberans: A review of the literature

Background: Dermatofibrosarcoma protuberans (DFSP) is a malignant fibrohistiocytic neoplasm that is slow-growing, has low metastatic potential, and is locally infiltrative with a predisposition for recurrence. The development of DFSP can occur spontaneously, but anecdotal evidence suggests a correlation between preceding injury and tumor onset.

Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Standard of Care and Treatment Recommendations from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG).

This paper describes the standard of care for patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) and the therapeutic recommendations developed by the European paediatric Soft tissue sarcoma Study Group (EpSSG). NRSTS form a very mixed group of mesenchymal extraskeletal malignancies. Their rarity, heterogeneity, and aggressiveness make the management of children and adolescents with these tumors complex and challenging. The overall cure rate for patients with NRSTS is around 70%, but survival depends on several prognostic variables, such as histotype and tumor grade, extent of disease and stage, tumor size, and tumor site. While surgery remains the mainstay of treatment for most of these tumors, a multimodal therapeutic approach including radiotherapy and chemotherapy is required in many cases. The EpSSG NRSTS 2005 study was the first prospective protocol tailored specifically to NRSTS. Together with the ARST0332 study developed by the North-American Soft Tissue Sarcoma Committee of the Children’s Oncology Group (COG), the EpSSG NRSTS 2005 study currently represents the benchmark for these tumors, establishing risk-adapted standards of care. The EpSSG has developed common treatment recommendations for the large group of adult-type NRSTS (including synovial sarcoma), and specific treatment recommendations for other particular adult-type histologies (ie, alveolar soft-part sarcoma, clear cell sarcoma and dermatofibrosarcoma protuberans); other highly malignant tumors with a biology and clinical behavior differing from those of adult-type NRSTS (ie, rhabdoid tumors and desmoplastic small round cell tumor); and soft tissue tumors of intermediate malignancy (ie desmoid-type fibromatosis, inflammatory myofibroblastic tumors, and infantile fibrosarcoma). New effective drugs are needed for patients whose NRSTS carries the worst prognosis, ie, those with unresectable tumors, metastases at diagnosis, or relapsing disease. Progress in this area relies on our ability to develop international integrated prospective collaborations, both within existing pediatric oncology networks and, importantly, between the communities of specialists treating pediatric and adult sarcoma.

Margin Size for Unique Skin Tumors Treated With Mohs Micrographic Surgery: A Survey of Practice Patterns.

Mohs micrographic surgery (MMS) has shown lower recurrence rates for unique skin tumors compared with wide local excision, but there is a lack of standardization on margin size. We aimed to assess MMS practice patterns of margin sizes for unique skin tumors. A survey was distributed to members of the American College of Mohs Surgery (ACMS). Demographic information on participants was collected in addition to initial/subsequent MMS margin size for unique skin tumors, including dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), melanoma, sebaceous carcinoma, microcystic adnexal carcinoma (MAC), poorly differentiated squamous cell carcinoma (SCC), and Merkel cell carcinoma. Eighty-seven respondents completed the survey (response rate <10%). Given that no guidelines exist on MMS margins for less commonly treated skin tumors, this study helps give Mohs surgeons perspective on current practice patterns for margin sizes. Mohs surgeons are more likely to take larger initial margins for these common skin tumors compared with BCCs or SCCs.

33159 Predictors of use of Mohs micrographic surgery in the treatment of dermatofibrosarcoma protuberans of the head and neck: An analysis of the National Cancer Database

Objectives: Dermatofibrosarcoma protuberans of the head and neck (DFSPHN) is a locally infiltrative sarcoma. Mohs micrographic surgery (MMS) has been associated with reduced recurrence and comorbidities over wide local excision (WLE). However overall survival remains high regardless. This study analyzes predictors of the use of MMS in the treatment of DFSPHN, its role as the SOC, and its widespread availability for patients.

Dermatofibrosarcoma Protuberans - Case Report and Review of Literature

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow-growing, locally aggressive soft tissue sarcoma of the skin. The most common age of presentation for this entity of dermis is the third to fifth decade of life, whereas the most common site of involvement is the trunk and lower extremities, accounting for a total of 70 to 90 percent of the cases. Head and neck regions account for 10 to 15 percent of cases with the involvement of the scalp being the most common presentation in this region of the body.[1]&#x0D; Although DFSP frequently shows local recurrence with the incidence of 20-50 percent of the cases, distant metastasis has been reported rarely.[2] So the cure rate in early diagnosed cases is high with a 10-year survival rate of 99.1 %, so prompt diagnosis of the entity is important along with awareness about the disease.[3] Here in this article, we are going to present a case of DFSP along with a review of the literature regarding the imaging characteristics of the disease.

Corrigendum: Dermatofibrosarcoma Protuberans Re-excision and Recurrence Rates in the Netherlands Between 1989 and 2016.

Abstract is missing (Corrigendum)

MORPHOLOGICAL AND IMMUNOHISTOCHEMICAL FINDINGS OF UNUSUAL ORAL MESENCHYMAL TUMORS

<h3>Objectives</h3> To evaluate the features of uncommon oral benign and malignant tumors of fibroblastic, myofibroblastic, and fibrohistiocytic origin to better understand the diagnostic challenges of these tumors. <h3>Study Design</h3> This study evaluated benign fibrohistiocytic tumor (BFH; <i>n</i> = 4), myofibroma (MF; <i>n</i> = 2), solitary fibrous tumor (SFT; <i>n</i> = 6), fibrosarcoma (FS; <i>n</i> = 2), dermatofibrosarcoma protuberans (DFSP; <i>n</i> = 1), and myofibroblastic sarcoma (MFS; <i>n</i> = 1) obtained from a diagnostic service. We carried out morphologic and immunohistochemical (αSMA, vimentin, desmin, CD34, Bcl2, S100, CD68, CD99) analyses. <h3>Results</h3> Morphologic analysis showed several features in tumor cells and stroma of the tumors (multinucleated cells, clear cells, hypercellular and hypocellular areas). Immunohistochemistry demonstrated that all cases were positive for vimentin and negative for desmin. BFH tumors exhibited immunopositivity for CD68 and S100, whereas MFs were positive for αSMA and SFTs for CD34, CD99, and Bcl2. Regarding malignant tumors, DFSP was positive for CD34 and Bcl2, and MFS was positive for αSMA and Bcl2, whereas FS was negative for most of the antibodies analyzed. Furthermore, malignant tumors exhibit a higher proliferation index (Ki-67 > 10%). <h3>Conclusions</h3> Considering their morphologic similarity, the use of immunohistochemistry is of great value to help in the differential diagnosis of these tumors.

ASO Author Reflections: Improving Management Strategies for Patients with Primary Dermatofibrosarcoma Protuberans (DFSP).

ASO Author Reflections: Improving Management Strategies for Patients with Primary Dermatofibrosarcoma Protuberans (DFSP).

Closure of a Large Scalp Defect Using External Tissue Expansion.

The limited laxity of the scalp and hair-bearing nature makes it difficult to optimally repair. Primary closure is limited to small defects, while healing by secondary intention requires underlying periosteum. Furthermore, the use of free flaps and skin grafting can lead to unfavorable cosmetic outcomes. This report describes a patient with a large parietal scalp dermatofibrosarcoma protuberans. Multiple resections resulted in a large scalp defect with exposed calvarium. Integra was placed over the exposed calvarium after each debridement and Negative Pressure Wound Therapy (NPWT) was applied. After granulation tissue formed, 2 DermaClose continuous external tissue expansion systems were installed. Following the application and tightening of the external tissue expanders, the wound was reduced in size, facilitating primary closure of the opposing edges. This process allowed for a large area to be covered with hair-bearing scalp with good cosmetic results.

Review of primary superficial soft tissue mesenchymal tumors of malignant or intermediate biological potential.

The majority of the sarcomas are deep in location, larger than 5cm in size, and heterogenous in imaging appearance. However, when sarcomas occur superficially, these typical features may be absent, failing to alert the radiologist to a malignancy and mimicking one of the more numerous benign superficial tumors that make up the bulk of soft tissue mass evaluations. This manuscript will discuss the current role of imaging in recognizing features concerning for superficial soft tissue sarcomas. Provided is an overview of the demographic, clinical, and imaging features of the most commonly encountered superficial soft tissue sarcomas, including undifferentiated pleomorphic, leiomyosarcoma, synovial sarcoma, liposarcoma, myxofibrosarcoma, dermatofibrosarcoma protuberans, epithelioid sarcoma, and angiosarcoma. A less common but primary superficial tumor, angiomatoid fibrous histiocytoma, is also reviewed as the diagnosis may be confounding both clinically and on imaging studies.

A Case of Primary Dermatofibrosarcoma Protuberans of the Lower Anterior Abdominal Wall Treated by Abdominoplasty

Dermatofibrosarcoma protuberans is a rare skin neoplasm with a high recurrence rate and a local invasive behavior. Excisional surgery is the mainstay of treatment of such tumors. Obtaining an adequate surgical safety margin is essential to ensure radical excision, however this may result in extensive skin and soft tissue defects that may require resurfacing. In this clinical case report, we describe a case of a large DFSP of the lower anterior abdominal wall, treated by abdominoplasty, we also perform a relevant scoping literature review on the topic.