title: Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity.
abstract: Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.
authors.affiliation: From the Department of Pathology, Yale University School of Medicine, New Haven, Connecticut.
authors.forename: Romulo
authors.initials: R
authors.lastname: Celli
authors.forename: Guoping
authors.initials: G
authors.lastname: Cai
mesh_terms: D015415:Biomarkers / Q000378:metabolism; D001859:Bone Neoplasms / Q000175:diagnosis; D003937:Diagnosis, Differential; D006801:Humans; D007150:Immunohistochemistry; D007668:Kidney / Q000378:metabolism / Q000473:pathology*; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D025202:Molecular Diagnostic Techniques; D009154:Mutation; D009363:Neoplasm Proteins / Q000235:genetics / Q000378:metabolism; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D011379:Prognosis; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology
chemical_list: D015415:Biomarkers; D009363:Neoplasm Proteins