Soft tissue sarcoma extremity

Prolapsed uterine tumors within the cervix or vagina, are attached to the uterine cavity by a soft tissue stalk. Malignant tumors and leiomyoma are the first diagnostic considerations for a prolapsed uterine mass with a visible stalk at MRI. This article describes 4 cases of patients who presented with large, necrotic prolapsed uterine tumors that were surgically confirmed and were diagnosed prospectively on the basis of MRI findings. Imaging, particularly MRI, plays a crucial role in the management of patients with prolapsed pedunculated uterine tumors, especially for pre-operative localization and surgical treatment...

BACKGROUND: Alveolar soft part sarcoma is a rare tumour of soft tissues, mostly localized in muscles or deep soft tissues of the extremities. In rare occasions, this tumour develops in deep tissues of the abdomen or pelvis. CASE PRESENTATION: In this case report, we described the case of a 46 year old man who developed a primary splenic alveolar soft part sarcoma. The tumour displayed typical morphological alveolar aspect, as well as immunohistochemical profile notably TFE3 nuclear staining...

Epithelioid sarcoma (ES) is an uncommon soft tissue sarcoma. It is usually located in the extremities and exceptionally in the neck. Its diagnosis constitutes a real challenge which is based on histology and immunohistochemistry staining that must be interpreted with caution given the anatomopathological similarities to other tumors. In this article, we report a case of a 37-year-old man admitted for a locally advanced ES of the neck. There were suspicions of lymph node metastases of nasopharyngeal carcinoma at the first pathological examination...

Malignant peripheral nerve sheath tumors (MPNST) are rare and aggressive soft tissue sarcomas. MPNST diagnosis is made based on biopsy, but distinct features are present on ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI). We present a case of a 24-year-old man presenting with abdominal pain and lower-extremity weakness found to have a large MPNST originating from the left femoral nerve and describe findings on imaging and their histopathologic correlation.

Ewing sarcoma (ES) is the second most common primary malignant bone tumour in children and adolescents. About 14.5% of primary malignancies develop in pelvic bones, where they typically have worse prognoses than extremity or acral sarcomas. It usually presents with aggressive features on radiology scans, but may also present with different radiological characteristics. In this series, we describe rare appearances of pelvic skeletal Ewing sarcoma, with large extraosseous cystic component on imaging, defined by the presence of fluid-filled spaces in the extraosseous tumour lesion, which distinguishes it from the solid nature of conventional ES...

BACKGROUND: The reconstructive ladder relies mostly on defect size and depth to determine reconstructive technique, however, in actuality, many more variables ultimately inform reconstructive decision making, especially regarding extremity soft tissue sarcoma (eSTS) defects. The purpose of this study was to describe eSTS patients who will most optimally benefit from an advanced method of reconstruction (defined as a pedicled regional flap or free flap) and to create a simple risk assessment scale that can be employed in clinical practice...

BACKGROUND: Patients with neurofibromatosis type I (NF1) have an increased risk of developing soft-tissue sarcomas, particularly those related to the nervous system. Epithelioid sarcoma (ES) is an exceptionally rare subtype of soft-tissue sarcoma, with limited knowledge about its clinical presentation and optimal management in NF1. This report aims to provide insights into the characteristics and outcomes of ES in NF1 patients. CASE DESCRIPTION: A 37-year-old man with a history of NF1 presented with a progressively worsening mass on his right inner thigh...

Soft tissue sarcomas (STS) of the extremity and trunk are heterogeneous and rare tumors that require coordinated multidisciplinary management. Surgical resection remains the backbone of treatment for localized tumors, with the addition of radiotherapy to surgery to achieve high rates of local control. Despite this, overall survival is limited because of significant distant metastatic risk and a lack of efficacious systemic therapies. Clinical trials have produced conflicting results on the impact of systemic therapy in the neoadjuvant and adjuvant settings for patients with localized disease, leaving systemic treatment decisions largely guided by shared decision making and prognostic prediction tools such as nomograms...

Ewing sarcoma is the second most frequent primary bone tumour of childhood and adolescence. The aim of this report is to describe the imaging, pathology, clinical findings, and treatment of a primary intradural extramedullary Ewing sarcoma with a unique intracranial metastatic component in a pediatric patient. A 14-year-old girl with a history of mood disorders presented to the emergency department with a 3-week history of neck torticollis, cervical pain, paresis, and paresthesia of the upper and lower extremities on the left side...

Malignant peripheral nerve sheath tumour (MPNST) is an aggressive soft tissue sarcoma with a poor prognosis, affecting most commonly the extremities. The lungs constitute the most frequent location for distant metastases. Half of all MPNSTs arise in patients with neurofibromatosis type 1, while approximately 10% are radiation induced and the rest are sporadic.The authors present a pregnant woman in her 40s with a sporadic MPNST of the lower limb and with lung metastases at diagnosis. Treatment consisted of interilioabdominal amputation, followed by adjuvant chemotherapy...

BACKGROUND: Undifferentiated pleomorphic sarcoma (UPS) is a relatively rare but aggressive neoplasm. We sought to utilize a multi-institutional US cohort of sarcoma patients to examine predictors of survival and recurrence patterns after resection of UPS. METHODS: From 2000 to 2016, patients with primary UPS undergoing curative-intent surgical resection at seven academic institutions were retrospectively reviewed. Epidemiologic and clinicopathologic factors were reviewed by site of origin...

BACKGROUND: While the list of fusion-driven soft tissue neoplasms is expanding rapidly, their importance among cutaneous and superficial mesenchymal and adnexal neoplasms remains poorly understood. This challenge is especially evident in cases with ambiguous histopathology that are difficult to classify based on morphology. AIMS: Our goal was to investigate the benefits of next-generation sequencing in diagnosing complex cutaneous neoplasms. MATERIALS & METHODS: Departmental archives were searched for fusion-driven cutaneous neoplasms...

Folliclular dendritic cell sarcoma (FDCS) is an extremely rare neoplasm originating from folliclular dendritic cells, both nodally and extranodally. Its primary presentation as a large colonic mass is rare and can be misdiagnosed as epithelial tumor/soft tissue tumor both clinically and through histomorphology. Due to its rarity and limited consensus guidelines about its management, it presents as a diagnostic and therapeutic challenge for pathologists and oncologists. However, accurate diagnosis is imperative due to its distinct prognostic and therapeutic implications...

BACKGROUND: Treatment options for patients with metastatic sarcoma are limited. The goal of this study was to investigate the effectiveness of temozolomide in pretreated patients with soft tissue sarcoma. METHODS: We recorded the pathological, clinical, and treatment data of the patients with metastatic soft tissue sarcoma retrospectively. We evaluated the efficacy and side effects of temozolomide in this patient group. RESULTS: This study involved 16 patients...

INTRODUCTION: Sclerosing epithelioid fibrosarcoma (SEF) is an extremely rare form of bone and soft tissue sarcoma. It occurs mainly in the deep soft tissue of the lower extremities, with few cases reported in the head and neck region. Tumors involving the oral and maxillofacial region (OMFR) and intraosseous examples are rare. CASE PRESENTATION: We present a 52-year-old male with a radiolucent lesion at the apex of the left mandibular second molar tooth with the clinical impression of a periapical granuloma...

INTRODUCTION AND IMPORTANCE: Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma (MFH), is a highly aggressive soft tissue sarcoma characterized by its pleomorphic histology and lack of differentiation. CASE PRESENTATION: A 35-year-old man visited our oncology department with a complaint of a growing mass in his left buttock area. The mass had been increasing in size for the past six months, affected by local and systemic infection...

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Soft tissue sarcomas form 1% of all cancers and are rare. The lower limb is one of the commonest sites of sarcoma, with the thigh accounting for the majority of these tumors. Large tumors abut the neurovascular bundles both anteriorly and in the hamstring compartment. Nerve involvement, especially the major nerves such as the femoral and the sciatic, by these tumors, was considered to be an absolute contraindication for limb salvage procedures. We present our data of major nerve resection without amputation, in an attempt to demonstrate the possibility of equivalent functional and oncological outcomes in these rare tumors...

Soft tissue sarcomas (STS) are a diverse group of malignant mesenchymal neoplasm.But, due to their low prevalence, very little data is available worldwide as well as in India regarding their clinico-epidemiological pattern.In this study we analysed the clinico-demographic profile of STS patients attending a tertiary care centre of Eastern India over the last 5 years. We analyzed the prospectively maintained database of a tertiary care centre of West Bengal in Eastern India, and collected the data regarding the demography, clinical profile, pathology and treatment of STS patients who attended our OPD between 2017-2021...

Synovial sarcoma (SS), a rare subtype of soft-tissue sarcoma distinguished by expression of the fusion gene SS18-SSX, predominantly affects the extremities of young patients. Existing anticancer drugs have limited efficacy against this malignancy, necessitating the development of innovative therapeutic approaches. Given the established role of SS18-SSX in epigenetic regulation, we focused on bromodomain and extra-terminal domain protein (BET) inhibitors and epigenetic agents. Our investigation of the BET inhibitor ABBV-075 revealed its pronounced antitumor effects, inducing G1-phase cell-cycle arrest and apoptosis, in four SS cell lines...