Dermatofibrosarcoma

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...

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is the most common sarcoma of the skin. Although distant metastases are infrequent, DFSP is highly aggressive locally with frequent local recurrences. It has been reported that the presence within the tumour of areas histopathologically mimicking fibrosarcoma may increase the risk of recurrence. OBJECTIVE: To review the clinical features of our patients with DFSP and the factors associated with recurrence of the tumour, focusing on the presence of fibrosarcomatous areas...

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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...

The utility of pedicled latissimus dorsi kiss flap for the reconstruction of chest wall defect is still an underutilized option. But the peculiar design and structure of the kiss flap with two semicircular flaps of equal diameter with same vascular trunk makes it amenable to cover large chest wall defects especially at places where plastic surgery facility is unavailable. We have used this flap to reconstruct large chest wall defect of size 20×18 cm in our patient operated for recurrent chest wall dermatofibrosarcoma protuberans...

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...

[This retracts the article DOI: 10.1155/2022/5509129.].

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...

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a rare soft-tissue tumor typically located in the trunk. We report a unique case of DFSP in the right inguinal region of an adult presenting with chronic lymphedema. Only 1 case of DFSP and chronic lymphedema association has been previously reported in the literature. Since we could not provide adjuvant radiotherapy (RT), we conducted an extensive review of its application in similar cases, exploring various surgical treatments. CASE REPORT A 42-year-old Cameroonian man with unexplained chronic lymphedema presented with a tumor in the inguinal region of the affected limb...

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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...

Low-grade myofibroblastic sarcoma (LGMS) is a rare spindle cell tumor with indolent course. Due to rarity and low-grade histologic features of LGMS, accurate diagnosis is challenging. We report a 63-year-old female patient with a three-month history of a 3.1 cm×2.5 cm sized, firm, skin-colored, painless, protruding left back mass. Initial excisional biopsy was performed and the mass was diagnosed as nodular fasciitis. After 18 months after excision, the mass recurred with pain and grew larger. Considering the clinical manifestations, diagnostic impression was changed as dermatofibrosarcoma protuberans not nodular fasciitis...

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...

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade sarcoma with locally infiltrative characteristics. A wide excision is made with a 5 cm gross margin. However, recently, Mohs micrographic surgery has emerged as an alternative. In this study, we evaluate a novel method (Saturn's Rings technique) for removing tumors that combines the advantages of wide excision and Mohs micrographic surgery. Between September 2001 and March 2020, 29 patients with DFSP, excluding the head and neck, were treated using Saturn's rings technique...

Connexins play a crucial role in the formation of gap junctions that connect cells to each other, as well as cells to the surrounding environment. In recent years, connexin 43 has been extensively studied in various human tumors. In this study, we conducted an immunohistochemical analysis to evaluate the expression of connexin in 16 dermatofibromas (DFs) and 13 dermatofibrosarcoma protuberans (DFSP). Connexin was diffusely expressed in the cytoplasm of all DFs with moderate or strong intensity, whereas all DFSPs showed negative staining...

BACKGROUND: Recent changes in the incidence and survival of dermatofibrosarcoma protuberans (DFSP) have not been described. OBJECTIVE: To characterize the incidence and survival of DFSP. MATERIALS AND METHODS: A retrospective cohort study of patients with DFSP from 2000 to 2020 in the Surveillance, Epidemiology, and End Results database was performed. Cox and Fine-Gray regression models were used to assess overall and DFSP-specific survival...

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...

Dermatofibrosarcoma protuberans (DFSP) is an atypical soft tissue malignancy that affects the dermis and subcutaneous tissue. The cause of DFSP is not clearly understood. This report highlights a rare case of DFSP of the left breast. We report a case of an 18-year-old female with past medical history of type 1 diabetes mellitus, who presented to the breast imaging clinic with a six-month history of left breast lump and associated skin discoloration. The patient had a dedicated left breast ultrasound which showed an indistinct, oval, hyperechoic mass in the superficial breast, measuring 1...

The reported complications' rate of perforator propeller flaps is variably high, but the etiology of distal flap necrosis, potentially linked to vascular insufficiency, is yet to be clarified. Vascular augmentation procedures have been previously described involving an extra anastomosis of a superficial vein, while a perforator-to-perforator supercharging approach has been only sporadically documented in literature. We present a case of perforator-to-perforator vascular supercharging of an extended dorsal intercostal artery perforator (DICAP) propeller flap to provide a salvage option for pedicled flap complicated by venous congestion...