Key result
Renal Ewing sarcoma/primitive neuroectodermal tumor is a rare, lethal entity requiring careful morphological, immunohistochemical, and molecular evaluation for accurate diagnosis and treatment.
Establishing the correct diagnosis of renal Ewing sarcoma/primitive neuroectodermal tumor using ancillary molecular tests is critical due to its dismal prognosis and specific treatment requirements.
Accurate diagnosis of renal Ewing sarcoma remains essential given its lethality; literature review leaves optimal therapy open.
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.
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Celli et al. (2016) conducted a review in Ewing sarcoma/primitive neuroectodermal tumor of the kidney. Renal Ewing sarcoma/primitive neuroectodermal tumor is a rare, lethal entity requiring careful morphological, immunohistochemical, and molecular evaluation for accurate diagnosis and treatment.
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