DFSP

Dermatofibrosarcoma protuberans (DFSP) of the breast is an infrequent soft tissue sarcoma that usually affects young to middle-aged women. Our case report describes a unique occurrence of DFSP of the breast in an adolescent girl, which was initially being managed as a keloid for 2 years under dermatology despite being refractory to treatment. Once the diagnosis of DFSP was confirmed through punch biopsy, our patient underwent surgical excision of the lesion under general anaesthesia. Our patient was at an increased risk of damage to the ductal system due to proximity of the lesion to the nipple-areolar complex, warranting the need for early recognition and treatment...

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...

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing soft tissue sarcoma, typically presenting as a cutaneous lesion. However, its occurrence in chronic wounds is infrequently documented, posing diagnostic and therapeutic challenges. This report details the case of a 59-year-old female with no significant medical history, presenting with a chronic, non-healing wound on the left shoulder, persisting for three years. Initially a small nodule, it progressed into an ulcerating lesion. Physical examination revealed a contracted scar with restricted shoulder mobility...

Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive soft tissue sarcoma of the skin. DFSP typically presents as an asymptomatic, firm plaque in its earliest stage, gradually enlarging over months to years. This case report delineates a singular instance involving a 66-year-old female patient previously diagnosed with and treated for DFSP. The individual presented with a recurrent history of hospital admissions, manifesting symptoms of loose stools, generalised weakness, and diminished appetite...

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...

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...

AIMS: Kinase fusion-positive soft tissue tumors represent an emerging, molecularly defined group of mesenchymal tumors with a wide morphologic spectrum and diverse activating kinases. Here, we present two cases of soft tissue tumors with novel LTK fusions. METHODS AND RESULTS: Both cases presented as acral skin nodules (big toe and middle finger) in pediatric patients (17-year-old girl and 2-year-old boy). The tumors measured 2 and 3 cm in greatest dimension...

Dermatofibrosarcoma protuberans (DFSP) is a rare spindle cell soft tissue sarcoma of the dermis and subcutaneous tissue. We present the fourth case of scrotal DFSP in the literature, identified in a 32-year-old male with schizophrenia. Wide surgical excision and radical orchiectomy were performed revealing an uninvolved testicle and DFSP of the scrotum. A unique challenge to this case was concurrent aortic dissection and schizophrenia. Social determinants of health are associated with delay in presentation and poor appointment compliance in patients with schizophrenia...

Dermatofibrosarcoma protuberans (DFSP) is a rare and infiltrative soft tissue tumor. Our report details a distinctive case of DFSP with pan-TRK positivity in the right nasal dorsum of a 46-year-old female. Histological analysis identified NTRK fusion gene involvement in this patient, detectable through pan-TRK immunostaining. The case underscores the significance of comprehensive management for pan-TRK-positive DFSP in challenging facial locations, indicating the potential efficacy of TRK inhibitors.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) has a high recurrence rate after resection. Because of the lack of specific manifestations, recurrent DFSP is easily misdiagnosed as post-resection scar. A few series have reported ultrasound findings of recurrent DFSP; moreover, the usefulness of contrast-enhanced ultrasound in differentiating recurrent DFSP has not been studied. OBJECTIVE: We investigated conventional and contrast-enhanced ultrasound in the differential diagnosis of recurrent DFSP and post-resection scar...

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...

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare and slowly growing soft tissue tumor and it is frequently misdiagnosed and mismanaged like more common masses. Therefore diagnostic delays are common and may result in challenging reconstructions. CASE PRESENTATION: We report the peculiar case of a 36-year-old patient with dermatofibrosarcoma protuberans of the right iliac fossa misdiagnosed as vascular malformation for over 30 years. Due to the delayed diagnosis resulting in a large tumor to be resected, surgical reconstruction was performed with a miniabdominoplasty approach with an excellent cosmetic and functional result...

Dermatofibrosarcoma protuberans (DFSP) is the most prevalent dermal sarcoma, characterized by the presence of the fusion of the collagen type I alpha 1 (COL1A1) gene with the platelet-derived growth factor beta chain (PDGFB) gene. Although PDGF receptor inhibitor imatinib mesylate was approved for the treating patients with unresectable or metastatic DFSP, disease progression was shown in 9.2% of the patients. Therefore, developing novel therapeutic strategies is crucial for improving the prognosis of DFSP...

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...

BACKGROUND: Harvesting an adequate-sized flap is challenging for reconstructing large defects on the abdominal wall. A subtotal thigh flap would be one of the choices as it provides a well-vascularized large flap with muscle components. Moreover, dermatofibrosarcoma protuberans (DFSP) is a low-grade dermal neoplasm with a high recurrence rate. There is still no consensus on the extent of resection to prevent a recurrence. OBJECTIVES: We present a case of a patient who underwent the reconstruction of a large abdominal wall defect with a subtotal thigh flap after the resection of recurrent DFSP...

BACKGROUND: Mesenchymal neoplasms of the uterus encompass a diverse group of tumors, with varying characteristics and origins, collectively accounting for 8% of uterine malignancies. The most common variants include uterine leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, adenosarcoma, and undifferentiated sarcoma. Clinical presentation is often nonspecific and can lead to delayed diagnosis. Uterine sarcomas are generally aggressive, resulting in poorer prognosis compared to carcinomas...

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant fibroblastic tumor. DFSP has an insidious onset, slow growth, and heterogeneous presentation that can create a delay in diagnosis and increase morbidity. In this case report, we present a child with DFSP that presented as a large, slow-growing mass over the dorsum of the left foot. She underwent successful surgical excision with no functional sequelae.

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...

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...