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Primary Ewing sarcoma of the kidney
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  • Published on:
    IHC with CD99 over expression and cytogenetic analysis like translocations involving the EWS locus EWSR1 gene rearrangement are required for definitive diagnosis of Primary Ewing sarcoma of Kidney which is very rare entity

    Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumours typically found in children and adolescents. These tumours belong to the family of small round cell tumours and are of neuro ectodermal origin Primary Ewing sarcoma (EWS) of the kidney is a rare tumor in adults. It was first described in 1975 by Seemayer and colleagues, and has since been sporadically documented in the literature Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are now considered part of a spectrum of neoplastic diseases now known as Ewing’s sarcoma family tumors (ESFT), which also includes other malignancies The ESFT are most common in bone. Extraskeletal ESFT are less common and can affect the skin, soft tissue, or viscera So Renal primary sarcomas are a rare group of renal tumours. Ewing sarcoma/PNET of the kidney is distinctly rare, with more than 100 cases reported globally. Among these, leiomyo sarcoma is the most common (40–60%) followed by lipo sarcoma (10–15%) Sources of renal EWS include neural cells that invaginate into the kidney during its development some authors theorize that embryonic neural crest cells migrate into the kidney and undergo tumorigenesis It is primarily a genetic disease with case...

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    Conflict of Interest:
    None declared.