Teratoid Wilms Tumor: Report of a Rare Histologic Variant With Review of Literature

Wilms tumor (WT) is the most common pediatric renal neoplasm, and Teratoid Wilms' tumor (TWT) is a rare histologic variant of WT, which consists predominantly of well-differentiated heterologous mesenchymal and/or epithelial elements. We report a case of TWT in a toddler who presented with an incidentally detected abdominal lump. Trucut biopsy was suggestive of WT with rhabdomyomatous differentiation. Six weeks of neoadjuvant chemotherapy was given with minimal response. Radical nephroureterectomy with lymph node sampling was performed and histopathology was suggestive of TWT. The child is asymptomatic and disease-free at follow-up.