Ewing\'s sarcoma (EWS) is a highly aggressive malignant bone tumor primarily affecting adolescents and young adults. Despite the efficacy of chemoradiotherapy in some cases, the cure rate for patients with metastatic and recurrent disease remains low. Therefore, there is an urgent need for innovative therapeutic approaches to address the challenges associated with EWS treatment. Epigenetic regulation, a crucial factor in physiological processes, plays a significant role in controlling cell proliferation, maintaining gene integrity, and regulating transcription. Recent studies highlight the importance of abnormal epigenetic regulation in the initiation and progression of EWS. A comprehensive understanding of the intricate interactions between EWS and aberrant epigenetic regulation is essential for advancing clinical drug development. This review aims to provide a comprehensive overview of both epigenetic targets implicated in EWS, integrating various therapeutic modalities to offer innovative perspectives for the clinical diagnosis and treatment of EWS.

OBJECTIVE: Cold physical plasma (CPP) has emerged as an effective therapy in oncology by inducing cytotoxic effects in various cancer cells, including chondrosarcoma (CS), Ewing\'s sarcoma (ES), and osteosarcoma (OS). The current study investigated the impact of CPP on cell motility in CS (CAL-78), ES (A673), and OS (U2-OS) cell lines, focusing on the actin cytoskeleton.
METHODS: The CASY Cell Counter and Analyzer was used to study cell proliferation and determine the optimal concentrations of fetal calf serum to maintain viability without stimulation of cell proliferation. CellTiter-BlueCell viability assay was used to determine the effects of CPP on the viability of bone sarcoma cells. The Radius assay was used to determine cell migration. Staining for Deoxyribonuclease I, G-actin, and F-actin was used to assay for the effects on the cytoskeleton.
RESULTS: Reductions in cell viability and motility were observed across all cell lines following CPP treatment. CPP induced changes in the actin cytoskeleton, leading to decreased cell motility.
CONCLUSIONS: CPP effectively reduces the motility of bone sarcoma cells by altering the actin cytoskeleton. These findings underscore CPP\'s potential as a therapeutic tool for bone sarcomas and highlight the need for further research in this area.

Ewing sarcoma is one of the small round blue cell tumors of childhood that typically affects bone. Recently, a subgroup of undifferentiated round-cell sarcomas has been genetically identified as BCOR (B-cell Line 6 Corepressor)-altered sarcomas (BAS). We present a case of a six-year-old male child who presented with a chief complaint of shortness of breath and tachypnea and was found to have a large mediastinal mass concerning sarcoma. Preliminary biopsy results were positive for small round blue cells, possibly Ewing sarcoma. After six cycles of chemotherapy, with subsequent shrinkage of mediastinal mass, the patient was able to undergo wedge resection and excision of the mass with en bloc resection of the fifth and sixth rib, preserving his right lung. Final tissue pathology was positive for BAS. There have been only four reported cases of BAS of the chest wall and zero reported cases of primary tumor presentation of the lung, making this a rare presentation of the disease.

UNASSIGNED: Ewing\'s sarcoma is the second most common primary malignant bone tumor in children and adolescents, after osteosarcoma. It is a rare tumor, with the axial skeleton being the preferred site of development, followed by the long bones. Diagnosis is evoked by imaging and confirmed by histology. Treatment is based on intensive chemotherapy with local surgical treatment in operable forms, and in some cases, radiotherapy. There are only a few sporadic cases in the literature describing distal fibular localization.
UNASSIGNED: We report the case of a 7-year-old child who presented with pain on the lateral aspect of the left ankle without local inflammatory signs. Radiological findings revealed a metaphyseal-diaphyseal osteolytic tissue process of the left fibula, and histology concluded that it was Ewing\'s sarcoma. We proceeded with neoadjuvant polychemotherapy followed by segmental resection of the distal fibula with an anteroexternal tibial rod, plus tibiotalar, and talocalcaneal arthrodesis without recourse to radiotherapy.
UNASSIGNED: The management of Ewing\'s sarcoma is constantly evolving. Its distal fibular location in a growing limb makes it even more difficult. It must be personalized, multidisciplinary, and carried out in specialized centers.

Ewing\'s sarcoma of the kidney is a rare tumor. Although renal carcinomas are known to involve the inferior cava, extension of the tumor up to the right atrium is not common. In the majority of cases when the tumor extends into the infrahepatic part of the inferior vena cava, it can be removed from the abdominal approach. Few patients require the use of cardiopulmonary bypass for removal of the tumor in the inferior vena cava and right atrium. The management of patients requiring resection of kidney tumors and right atrial mass is more complicated and requires a team approach consisting of oncosurgeons, cardiac surgeons, and cardiac anesthetists. The resection of the kidney tumor with a mass in the right atrium is usually done concomitantly. The cardiopulmonary bypass cannulation strategy needs to be modified in such cases.

Ewing\'s sarcoma is a rare and highly aggressive bone tumor primarily affecting children and adolescents. It commonly presents in the pelvic and axial skeleton, with sacral involvement posing unique challenges due to its intricate anatomical location. This report details the case of an 18-year-old male with sacral Ewing\'s sarcoma, emphasizing the diagnostic, surgical, and reconstructive aspects of management. The patient presented with lower back pain, lower limb weakness, and urinary incontinence, which prompted an extensive diagnostic evaluation. Magnetic resonance imaging and computed tomography scans revealed a large lytic mass extending from the S2 vertebra to the coccyx invading the presacral space. Biopsy confirmed the diagnosis of Ewing\'s sarcoma, characterized by the EWS-FLI1 type 1 translocation. A multidisciplinary team comprising neurosurgeons, colorectal surgeons, and plastic surgeons was formulated. En bloc resection of the tumor, lumbopelvic fixation, and soft-tissue reconstruction using bilateral gluteus maximus advancement flaps were successfully performed. The procedure aimed to address both the oncological and functional aspects of the patient\'s condition. Chemotherapy and radiotherapy were administered as adjuvant therapies. At 2-year follow-up, the patient was ambulating independently with no residual tumor on imaging. This case highlights the complex nature of sacral Ewing\'s sarcoma and underscores the importance of a multidisciplinary approach. The described surgical technique, including the innovative use of gluteus maximus advancement flaps for soft-tissue reconstruction, contributes to reducing wound complications and promoting successful patient outcomes. The presented approach serves as a valuable addition to the armamentarium of treatment options for this challenging malignancy.

UNASSIGNED: One of the most challenging diagnostic categories in the sinonasal tract includes small-blue-round-cell tumors. These are malignant tumors which show many overlapping histomorphology and immunohistochemistry (IHC) findings. Limited, small biopsy of these not completely excisable tumors adds to the diagnostic confusion.
UNASSIGNED: A cross-sectional study was done for 2 years (January 2018-December 2020) in a tertiary care institute, which included 70 cases of tumors of which 49 cases were malignant. All paraffin-embedded blocks were subjected to hematoxylin and eosin stain and IHC followed by molecular study wherever needed.
UNASSIGNED: Of the total cases, small-blue-round-cell tumor constituted the major category comprising 20 rare and interesting cases which included sinonasal undifferentiated carcinoma (4 cases), malignant lymphoma (2 cases of diffuse large B-cell lymphoma and 2 cases of extranodal natural killer/T-cell lymphoma), rhabdomyosarcoma (2 cases), olfactory neuroblastoma (2 cases), malignant melanoma (2 cases), plasmacytoma (2 cases), atypical Ewing\'s sarcoma (EWS) (1 case), EWS (1 case), nuclear protein in testis (NUT) carcinoma (1 case), and small-cell neuroendocrine carcinoma (1 case).
UNASSIGNED: Tumors of the sinonasal tract are very diverse, more so in small-round-cell tumor which present with a undifferentiated morphology. Thus, accurate diagnosis needs clinicoradiological parameters and special ancillary techniques such as IHC and molecular study in addition to histopathology for early diagnosis and therapy to prevent significant morbidity and mortality caused in these tumors.

UNASSIGNED: The study aims to categorize malignant small round cell tumors (MSRCTs) originating in various sites of the body with the objective of utilization of cytomorphological features and ancillary techniques.
UNASSIGNED: It is a cross-sectional study conducted over a time span of 3 years (2017-2020). 33 cases of tumors with round cell morphology were evaluated by fine needle aspiration cytology (FNAC).
UNASSIGNED: The application of cell block preparation supported by immunohistochemistry aided in the categorization of 23 cases with definite diagnosis and the rest were reported as MSRCTs.
UNASSIGNED: Among the categorized 23/33 cases, the most common diagnosis was Ewing\'s sarcoma (7/23) followed by 6 cases of lymphoma. There were 2 cases each of rhabdomyosarcoma and Langerhans cell histiocytosis (LCH) and 1 case each of neuroblastoma, desmoplastic small round cell tumor (DSRCT), myeloid sarcoma, neuroendocrine tumor of pancreas, plasmacytoma, and small cell carcinoma. Histopathology confirmation was available in 24/33 cases. Among the categorized tumors (23/33), biopsy correlation was available in 19 cases, of which concordant result was seen in 17 cases (89.47%), which were 6 cases of lymphoma, 5 cases of Ewing\'s sarcoma (EWS), 2 of rhabdomyosarcoma, and 1 each of neuroblastoma, small cell carcinoma, DSRCT, and LCH. Discordant result was seen in one case of rhabdomyosarcoma and a case of synovial sarcoma reported as extraskeletal EWS in cytology. Out of the uncategorized cases reported as MSRTCs, histopathology was available in 5 cases which were diagnosed as rhabdomyosarcoma (1 cases), lymphoma (1 case), amelanotic melanoma (1 case), and extraskeletal EWS (2 cases).
UNASSIGNED: Categorization of MSRCTs should be done to implement appropriate therapeutic protocol. FNAC provides a rapid diagnosis contributing immensely for the timely management of the patient. Detailed cytomorphological evaluation serves as a guide for further evaluation by ancillary techniques leading to definitive diagnosis.

Data on gonadotoxicity of chemotherapies are essential to better counsel young females and males about the risk of infertility and to better indicate fertility preservation measures before cancer therapies. However, such data have not recently been reviewed for bone cancer. Therefore, a systematic literature search was conducted considering papers published since 2000. This study is part of the FertiTOX® project, which aims to improve the lack of data regarding gonadotoxicity of cancer therapies to enable more accurate counseling regarding fertility preservation. Only relapse-free women and men were included. Gonadotoxic therapy-induced suspected infertility was defined as very low anti-mullerian hormone, high gonadotropin concentration, amenorrhea, oligomenorrhea, azoospermia, or oligozoospermia. The quality of the individual studies was assessed using the Newcastle-Ottawa Scale (NOS). In total, 11 out of 831 studies were included in the review. Suspected infertility was found in 10/190 (5.1%, range 0%-66%) of female patients with osteosarcoma (six studies), in 24/46 (52.2%, range 46%-100%) of male patients with osteosarcoma (three studies), in 18/138 (13.0%, range 3%-18%) of female patients with Ewing\'s sarcoma (three studies), and in 34/38 (89.5%) of male patients with Ewing\'s sarcoma (one study). A risk calculation in relation to specific chemotherapies was not possible. Risk of suspected infertility tends to be higher in Ewing\'s sarcoma in which all patients received chemotherapies with alkylating agents. Two of the 11 included studies received a high NOS quality score, whereas the remaining nine studies received a low quality score, mainly because of the lack of a comparator group. Published data are too limited for precise estimation of the gonadotoxicity. However, data indicate clinically relevant risk for infertility, supporting counseling patients before chemotherapy about fertility preservation measures.

UNASSIGNED: Ewing sarcoma (ES) is a malignant and aggressive bony tumor affecting the most common age group of 5-20 years. It constitutes 10%-15% of all bone sarcomas and is the second most common primary malignant bone tumor after osteosarcoma. It usually presents with pain, which is typically constant and progressive in nature. The primary source of pain is due to the instability of the spine to support the weight of the body, the vertebral body\'s expanding cortices due to the growing mass, compression of nerve roots due to tumour mass, pathologic fractures, spinal cord compression, and invasion of tissue by the tumour mass.
UNASSIGNED: We reviewed the literature on Ewing\'s Sarcoma of the spine to evaluate its etiology, clinical presentations, differential diagnosis, imaging modalities and management with chemotherapy, radiotherapy, and surgical management. PubMed, EMBASE, Google Scholar and Cochrane key articles were searched. Keywords like \'Ewing\'s Sarcoma,\' \'Spine,\' \'etiology,\' \'treatment,\' \'surgical management,\' and \'en bloc resection\' were used.
UNASSIGNED: The current management of Ewing\'s sarcoma of the spine usually involves three primary modalities: combination chemotherapy, surgery and/or radiotherapy. Recent improvements in combination chemotherapy (vincristine, doxorubicin, cyclophosphamide +/- Ifosfamide and etoposide) are among the most significant factors for improving survival. Also, recent advancements in radiotherapy, instrumentation, and fusion techniques in surgical management have been demonstrated to improve local disease control and overall survival.
UNASSIGNED: Primary Ewing sarcoma of the spine is a rare condition affecting the most common age group of 5-20 years, accounting for 1-3 cases/million/year. About 5 % of cases have spine involvement. Recent improvements in combination chemotherapy have improved the overall survival rates. Enbloc resection and/or radiotherapy have improved local control of the disease.