Ewing sarcoma is a rare but aggressive disease most common in young adults. This cancer is driven by a unique chimeric fusion oncogene but targeted strategies have been elusive. Here we report the identification of the protein kinase PKC-ß (PRKCB) as a disease-specific druggable target for treatment of Ewing sarcoma. We found that transcriptional activation of PRKCB was directly regulated by th...

The occurrence of Ewing sarcoma as a secondary malignancy is an extremely rare event in long-term cancer survivors. In addition, the occurrence of Ewing sarcoma in the adrenal gland is highly unusual. In this case report, we treated a 20-year-old male patient with cyclophosphamide, doxorubicin, vincristine, dexamethasone, and methotrexate and cytarabine chemotherapy following a diagnosis of Sta...

PURPOSE Despite advances in therapy, >50% of patients with Ewing sarcoma will relapse. The current prognostic factors are not optimal for risk prediction. Studies have shown that telomere length could predict outcome in different malignancies. Our aim was to evaluate whether telomere length could be a better prognostic factor in Ewing sarcoma and correlate the results with clinical variables, o...

Numerous molecular abnormalities contribute to the genetic derangements involved in tumorigenesis. Chromosomal translocations are a frequent source of these derangements, producing unique fusion proteins with novel oncogenic properties. EWS/ETS fusions in Ewing sarcoma are a prime example of this, resulting in potent chimeric oncoproteins with novel biological properties and a unique transcript...

background: understanding the differences in genetics of malignancies is crucial for therapeutic decisions. this systematic review was conducted to evaluate the current evidence on genetics of bone tumors in the context of pediatric cancer. material and methods: we performed a systematic review of the literature published on genetics of pediatrics bone tumors, using pubmed, google scholar, scie...

Ewing sarcoma is the second most common pediatric bone tumor, with three cases per million worldwide. In clinical terms, Ewing sarcoma is an aggressive, rapidly fatal malignancy that mainly develops not only in osseous sites (85%) but also in extra-skeletal soft tissue. It spreads naturally to the lungs, bones, and bone marrow with poor prognosis in the two latter cases. Bone lesions from prima...

Ewing Sarcoma is the second most common primary bone cancer. To date, only a few cases of cutaneous have been published. We present unique case Sarcoma, which presented as blue to violaceous mass on young woman’s chest. The lesion clinically did not align with previous reports but pathology and immunohistochemical stains confirmed diagnosis. Because may masquerade benign tumors, dermatologists ...

Abstract Background Ewing sarcoma represents a spectrum of aggressive malignancies with the poor outcome. Primary renal is rare and accounts for less than 1% masses. Case presentation We present case 45-year-old male presented in OPD complaint right flank pain hematuria. He underwent contrast-enhanced CT abdomen which depicted mass liver lesions. Robotic Right Radical Nephrectomy pathological d...

In order to explore the potential of immune checkpoint blockade in sarcoma, we investigated expression and clinical relevance of programmed cell death-1 (PD-1), programmed death ligand-1 (PD-L1) and CD8 in tumors of 208 sarcoma patients. Primary untreated osteosarcoma (n = 46), Ewing sarcoma (n = 32), alveolar rhabdomyosarcoma (n = 20), embryonal rhabdomyosarcoma (n = 77), synovial sarcoma (n =...

Ewing sarcoma is the second most common bone malignancy in children and young adults. It is driven by oncogenic fusion proteins (i.e. EWS/FLI1) acting as aberrant transcription factors that upregulate and downregulate target genes, leading to cellular transformation. Thus, identificating these target genes and understanding their contribution to Ewing sarcoma tumorigenesis are key for the devel...