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Detection of Metastatic Tumor Cells in Bone Marrow
Published in Adrian P. Gee, BONE MARROW PROCESSING and PURGING, 2020
Autologous BMT has been used to treat Ewing’s Sarcoma.75 Bone marrow involvement, as identified by routine analysis, is known to exist in approximately 15% of these patients.76 Immunologic testing is not available, because there are no Ewing’s Sarcoma-specific antibodies. Most of the available antibodies also react with normal hematopoietic cells. Thus routine analysis remains the sole diagnostic test used to screen potential candidates.
Primary Bone Tumors
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Jeremy S. Whelan, Rob C. Pollock, Rachael E. Windsor, Mahbubl Ahmed
The discovery of a chromosomal translocation specific to Ewing sarcoma (t11;22) has provided important insights, particularly in identifying primitive neuro-ectodermal tumors which share this translocation as having a common lineage. These related diseases are now usefully referred to as the Ewing sarcoma family of tumors.
Primary bone tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Jennifer N Murphy, Steven L J James, Murali Sundaram, A Mark Davies
Ewing sarcoma is a highly aggressive, malignant tumour of childhood and adolescence. It is characterized by the presence of small round cells of uncertain origin which do not produce a tumour matrix (1). Ewing sarcoma can occur in any bone but most commonly occurs in the proximal meta-diaphyseal region of long bones, with the femur most commonly involved (21%). The extremities, ribs, and pelvis account for approximately 86% of cases (55). It typically presents between the ages of 5 and 30 years and is the second most common malignant primary bone tumour of childhood and adolescence. It accounts for approximately 6%–8% of primary malignant bone tumours. Approximately 80% of cases occur before the age of 20 years (1). Males are more frequently affected than females (1.4:1) and it is much more common in the Caucasian population than other ethnic groups. Metastatic disease is present in 20%–30% of patients at presentation (56).
Efficacy of the VIT (vincristine, irinotecan and temozolomide) regimen in adults with metastatic Ewing sarcoma
Published in Journal of Chemotherapy, 2023
Izzet Dogan, Ayca Iribas, Melin Aydan Ahmed, Mert Basaran
Our study was a retrospective analysis. Ethics committee approval was obtained from the local committee before the start of the study. Our study was conducted according to good clinical practice standards. The patients included in the study consisted of patients followed in a single oncology center between 2014 and 2020. Patients were identified through the hospital database. All patients had metastatic Ewing sarcoma who were previously treated with at least one series of chemotherapy, and received with the VIT regimen were included in the study. Patients who did not have sufficient data for statistical analysis were excluded from the study. The pathological and clinical features of the patients were recorded from the patient files. The chemotherapy regimens, radiotherapies, and surgical characteristics of the patients before and after the VIT regimen were noted.
Bone tumors effective therapy through functionalized hydrogels: current developments and future expectations
Published in Drug Delivery, 2022
Ruyi Shao, Yeben Wang, Laifeng Li, Yongqiang Dong, Jiayi Zhao, Wenqing Liang
Ewing sarcoma usually affects individuals in the initial 30 years of age and is characterized by round, small blue cells malignancy (Rajani & Gibbs, 2012). It is considered the second most frequently occurring malignant bone cancer in adolescents and children, with peak incidence at 15 years (Rajani & Gibbs, 2012; Thanindratarn et al., 2019; Tzanakakis et al., 2021). Adjuvant chemotherapy, local treatments (surgical or radiotherapy interventions), and neo-adjuvant chemotherapy are the conventional treatment approaches for such tumors. Surgical resection in healthy margins is preferred over radiotherapy alone (Thévenin-Lemoine et al., 2018). For localized Ewing sarcoma, the overall five-year survival rate is 70 to 80%, however, worse patient outcomes are observed in cases where large tumors, pelvic involvement, or incomplete tumor regression after chemotherapy is observed (Thanindratarn et al., 2019).
Targeting the DNA damage response in pediatric malignancies
Published in Expert Review of Anticancer Therapy, 2022
Jenna M Gedminas, Theodore W Laetsch
Ewing sarcoma is the second most common primary bone tumor of childhood and adolescence [80]. The tumor is defined by a chromosomal translocation fusing the EWSR1 gene to a member of the ETS family of transcription factors, most commonly FLI1. Outside of this translocation, the tumor has a very low mutational burden; however, the EWS-FLI1 transcription factor leads to the dysregulation of several genes involved in the response to DNA damage including CHK2, MAP4K2, ABL1, and ATM [81–83]. Ewing sarcoma is also known to have high levels of endogenous replication stress, making the cells highly reliant on the ATR pathway for the repair of the resultant DNA damage and maintenance of cell viability [29,84]. Gorthi et al. has shown that Ewing sarcoma cells have an increased accumulation of R-loops which are known to be associated with high levels of DNA damage [84]. This was associated with increased RNAPII binding at the same site as the R-loops, consistent with the knowledge that both EWSR1 and EWS-FLI1 regulate RNAPII elongation. Unresolved R-loops can lead to stalling of the replication fork, furthering the reliance on DNA damage repair [84].