Emerging, Uncommon, and Aggressive Pediatric Kidney Tumors: An Update and Diagnostic Pitfalls.
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Although favorable histology Wilms tumors are the most prevalent pediatric renal tumor, the remainder includes a small but diverse and important group of malignant neoplasms. Among these are diffuse anaplastic Wilms tumor, blastemal Wilms tumor, anaplastic sarcoma of kidney, and clear cell sarcoma of kidney. Given their rarity, these tumors may pose diagnostic challenges for pathologists, but accurate diagnosis is essential to ensuring these aggressive tumors are managed appropriately. This article summarizes the salient clinical, histopathological, and molecular features of these uncommon but important tumors.