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The differential diagnosis of renal tumors, particularly in adolescents, may be challenging. We describe an 11‐year‐old female with a primary intra‐renal mass. Initial differential diagnoses included primitive neuroectodermal tumor (PNET), desmoplastic small round cell tumor (DSRCT), and Wilms Tumor (WT). Extensive pathologic and molecular analysis on initial and relapsed tumor samples confirmed WT...
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