We report an uncommon case of primitive neuroectodermal tumor/ Ewing\'s sarcoma of the lumbar and sacral nerve plexus in a 17years old boy who presented with an intense pain in the lower back radiating to legs. Magnetic resonance imaging showed a soft tissue mass with thickening of lumbar and sacral spinal nerve roots (L5-S3 level), along with widening of the corresponding foramina. There was also posterior scalloping of L5/S1 vertebrae and invasion of the sacral bone. A Partial resection has been performed, and the ensuing histopathology confirmed the diagnosis of PNET/Ewing\'s sarcoma. MRI in conjunction with histopathology are the key to narrow down the differential diagnoses list. PNET of lumbosacral area remain scarce, and only few cases have been reported nowadays. Given to the aggressivity of these tumors, the prognosis is poor despite appropriate treatment.

BACKGROUND: Ewing sarcoma is a malignant round-cell tumor that primarily affects bones in children. It can also arise in extraosseous tissues, such as the lung, kidneys, and liver. The presentation symptoms of Ewing sarcoma may include cough, dyspnea, and chest pain.
METHODS: This report details the history of a 15-year-old Syrian boy with a previous diagnosis of Hodgkin lymphoma who presented with chronic shoulder pain. Imaging studies revealed an 80 mm mass in the apex of the left lung, which was confirmed through histopathological examination to be Ewing sarcoma following a computed-tomography-guided biopsy. The patient received multiple cycles of chemotherapy and subsequently underwent surgical resection of the remaining mass.
CONCLUSIONS: This case highlights the rare occurrence of Ewing sarcoma in the lung and the unusual clinical presentation of shoulder pain without other accompanying symptoms.

UNASSIGNED: Ewing sarcoma (ES), a rare malignancy, comprises whatever the age, 4-15% of all primary bone tumors. It represents 1% of all malignant tumors in children and is the fourth most common bone malignancy after myeloma, osteosarcoma, and chondrosarcoma.
UNASSIGNED: A 12-year-old boy came to the Oral Surgery Department of Bretonneau Hospital referred by his dentist with a rapidly evolving swelling in the left mandibula for 6 weeks, which was initially diagnosed as a facial cellulitis. Cone beam computed tomography (CBCT) showed a poorly defined, expansile, and osteolytic tumor on the left side of the mandible. Clinical and radiographic findings were in favor of an aggressive primitive bone tumor. A mandibular biopsy under general anesthesia was performed in the Department of Surgical Oncology at Institut Curie in Paris, revealing an ES.
UNASSIGNED: Mandibular ES can mimic dental infections when swelling is the main clinical manifestation, which can lead to a delayed diagnosis. A correlation between clinical, radiological, histopathological, and immunohistochemical with cytogenetics is needed to confirm the diagnosis. Moreover, smaller tumors have better survival.Dentists must therefore be aware of the clinical signs of ES in order to quickly refer patients to a specialized department.
UNASSIGNED: Bellut N, Lutz CM, Lesnik M, et al. Ewing\'s Sarcoma of Mandible: A Case Report with Review of Literature. Int J Clin Pediatr Dent 2024;17(2):187-190.

UNASSIGNED: Ewing sarcoma is a primary malignant tumor of bone and, to a lesser extent, soft tissues. Within oncological management, surgery with extensive local control and reconstruction is the most accepted option, however, the size, extension of the tumor, the age of the patient and distant involvement can make this option difficult.
METHODS: We present a clinical case of a 3-year-old infant with Ewing sarcoma in the proximal femur, who was managed with wide resection of the tumor plus proximal humerus allograft, which acted as a proximal femur for 2 years and then presented joint failure due to what was done was the application of a cemented stem over the remnant of the allograft to save the hip.
UNASSIGNED: In the presented case, similar to the report by Zoccali a malignant tumor in the proximal femur of a pediatric patient is documented. In these cases, where the distal femoral growth plate is disease-free, a reconstruction technique preserving the growth plate is proposed.
CONCLUSIONS: The technique of proximal femoral allograft with a proximal humeral graft is an acceptable reconstructive treatment alternative for young patients with Ewing sarcoma, especially when conventional treatment options are limited. This approach helps avoid limb-threatening surgeries such as amputation or rotationplasty, providing a viable and functional solution for limb salvage in these cases.

BACKGROUND: Ewing\'s sarcoma (ES) is a neuroectodermal tumor that typically occurs in the bones and soft tissues of children and young adults. Primary renal ES is rare; only a few cases and a small case series have been documented, and only four cases involved primary renal ES in older people (> 65 years old).
METHODS: Herein, we describe the radiological and pathological features of primary renal ES in an older person. A 76-year-old man complained of poor oral intake and was found to have a large cystic renal mass with indistinct margins on computed tomography. Ultrasound-guided biopsy revealed that the tumor contained small round blue cells. The patient underwent a right radical nephrectomy. The tumor cells showed diffuse membranous CD99, and nuclear friend leukemia integration 1 transcription factor and NK2 Homeobox 2. Fluorescence in situ hybridization revealed EWSR1 translocation. Postoperatively, 18F-fluorodeoxyglucose positron emission tomography revealed no evidence of metastasis. The patient was diagnosed with primary renal ES. Six months following the surgery, local recurrence and distant metastasis were observed. Primary renal ES is rare and often lethal in older individuals. The specific imaging findings are unknown, and treatment protocols have not been standardized.
CONCLUSIONS: This case report describes the radiological and pathological features of primary renal ES in an older person.

Primary extraosseous intracranial Ewing sarcoma (ES) is an extremely rare disease, limited to the pediatric population, that primarily originates in the skull. Here, we present an unusual case of adult Ewing\'s sarcoma originating from the brain parenchyma. The 50-year-old male patient visited our hospital with severe headache lasting 3 weeks. MRI presented 6.1×6.2×5.2 cm sized heterogeneously enhanced mass containing peritumoral edema in the right frontal lobe. The patient underwent right frontal craniotomy, at which time the gray and red masses adhered to the surrounding brain parenchyma. The mass was completely resected using neuronavigation and electrophysiological monitoring. Histopathological examination revealed ES-compatible findings of small round cell tumor and CD-99 positive membranous immunostaining. Next generation sequencing revealed translocation and fusion of EWSR1 and FLI1, consistent with a confirmed diagnosis of ES. Consequently, the patient underwent postoperative radiotherapy. The present case revealed adult primary intracranial ES arising from the frontal lobe. Although its etiology remains poorly understood, intraparenchymal ES should be included in the differential diagnosis of parenchymal brain tumors.

Primary pleural Ewing sarcoma is a rare type of Ewing sarcoma with only a few case reports identified in the literature. The condition is challenging to diagnose with deceiving symptoms and wide differential diagnosis. Diagnosis is confirmed with a combination of radiological and pathological assessment. Treatment is similar to other types of Ewing sarcoma with chemotherapy and surgery being the mainstay of treatment. We identify an unusual presentation of pleural Ewing sarcoma in a 31-year-old male with a mass extending into the right ventricular outlet causing rapid deterioration of the patient.

Ewing\'s Sarcoma (ES) is an rare, small round-cell sarcoma that predominantly occurs in children and young adults, with both skeletal and extraskeletal manifestations. However, pancreatic ES, due to its rarity, is infrequently featured in scholarly literature, with only a scant 43 reported instances. Our study describes a case of pancreatic ES in an 8-year-old boy who was found to have an abdominal mass. Following an exhaustive examination, the boy was diagnosed with a neoplasm in the pancreatic head and underwent a complex surgical procedure encompassing pancreatoduodenectomy and partial transverse colectomy. Immunohistochemical assays confirmed the neoplastic cells\' positivity for Cluster of Differentiation 99(CD99), Vimentin, and NK2 Homeobox 2(NKX2.2), while genomic testing identified an EWSR1-FLI1(Ewing Sarcoma Breakpoint Region 1-Friend Leukemia Integration 1) gene fusion. This led to a conclusive diagnosis of pancreatic Ewing\'s Sarcoma. The patient underwent seven cycles of adjuvant chemotherapy, alternating between VDC (Vincristine, Doxorubicin, Cyclophosphamide) and IE (Ifosfamide, Etoposide) tri-weekly, but did not undergo radiotherapy. At present, the patient remains neoplasm-free. Through our case analysis and comprehensive review of the existing literature, we aim to underscore th rarity of pancreatic Ewing\'s sarcoma and to highlight the efficacy of our individualized therapeutic approach.

BACKGROUND: Primary chest wall tumors arise from muscle, fat, blood vessels, the nerve sheath, cartilage, or bone of the chest wall. One of the chest wall sarcomas is Ewing Sarcoma (ES), first described in 1921 by James Ewing, which is a highly aggressive bone and soft-tissue cancer. This case report aimed to present an Ewing Sarcoma with intra thoracic and multiple extra thoracic metastases in young adult male patient.
METHODS: We describe a unique case of metastatic of ewing\'s sarcoma in a 23-year-old male that showed a mass on the right lower posterior lung with pleural effusion, which was initially thought to be lung tumor that metastasized to the pleura. A thoracic CT scan showed a lobulated soft tissue mass on the right posterolateral thoracic wall, or pleura, with an expansion of soft tissue mass on the rib. Thoracal MRI showed tumor in the posterior right lower thoracic wall area, metastases of the left lateral rib, and right pleural effusion with atelectasis in the right inferior lobe of the lung. The patient also underwent a bone scan, scheduled for palliative radiotherapy and chemotherapy, and consulted to oncology surgeon.
CONCLUSIONS: Ewing sarcoma is a small, round, blue-cell mesenchymal malignancy. ES mainly affects children, adolescents, and young adults, with >1.5 cases per million children. Males are slightly more affected than females (sex ratio of 3:2). The definitive diagnosis requires biopsy proof (achieved by fine needle or core biopsy). The most common regions of metastasis are the lungs, pleural cavity, skeletal system, bone marrow, or combinations of these.
CONCLUSIONS: The 5-year survival rate is approximately 70 % when there is no metastasis; this rate falls to around 30 % when metastasis is present.

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. We report 3 cases of cystic presentation of ES, with imaging features supporting diagnosis of a primary malignant bone tumour arising from the superior pubic ramus with associated massive intrapelvic solid and cystic mass. CT-guided biopsy provided diagnosis of ES, with large intrapelvic soft tissue and cystic component. These patients underwent neo-adjuvant chemotherapy and proton beam therapy with significant reduction in size of the solid components, while the cystic components remained relatively unchanged. Two patients underwent surgical resection of the tumour (navigated P3 internal hemipelvectomy and hemipelvis P2/P3 resection, respectively), and one patient died while on treatment. In both who underwent surgery, histology showed ES with margins clear and more than 99% of treatment-induced necrosis. To the authors\' knowledge, this unusual presentation of pelvic ES is described for the first time in the literature as a case series, with particular reference to atypical extraosseous cystic changes, along with the clinical and radiological characteristics, and their treatment.