Introduction: Wilms tumor is the most common renal malignancy in children. The occurrence of Wilms tumor with various congenital genitourinary anomalies has been reported, particularly in horseshoe kidneys, hypospadias, disorder of sexual development, and double collecting system. However, Wilms tumor with crossed renal ectopia is a rare finding.
Case Description: We are reporting a case report of Wilms tumor in a 3-year-old girl who presented with a huge left flank mass with cross-fused renal ectopia. After the initial workup and triphasic computed tomography (CT) scan of the chest, abdomen, and pelvis for confirmation of diagnosis and metastasis, the patient underwent image-guided tissue biopsy followed by neo-adjuvant chemotherapy, left radical nephrectomy with separation of fused right ectopic renal moiety and adjuvant chemoradiation.
Practical Implication: This report shows an association of Wilms tumor with cross-fused renal ectopia, a rare combination. Unusual findings on imaging with unilateral flank mass should be considered as part of the differential diagnosis for this rare finding. Adjuvant chemotherapy and modern imaging helped delineate the anatomy and ease the surgery for safe resection, improving the overall outcome.