Abstract

Primitive neuroectodermal tumor (PNET) is a malignant small round cell tumor that typically arises from bone or soft tissue in adolescents and young adults. Renal PNET is extraordinarily rare and exhibits highly aggressive biological behavior with poor prognosis. It generally occurs in young adults and only a few pediatric cases have been reported. Here, we present a case of renal PNET in an 11-month-old boy which developed brain metastasis after two years, despite combined modality treatment.

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