Pediatric renal rhabdoid tumor: Imaging features and diagnostic challenges of a rare and aggressive neoplasm

The renal rhabdoid tumor is an extremely rare pediatric tumor, accounting for approximately 2% of renal tumors in children. It occurs in 80% of cases before the age of 2 years and is considered the most aggressive malignant renal tumor, with a particularly poor prognosis. It must be distinguished from Wilms tumor, the most common pediatric renal tumor. The case we report is characterized by a radiological presentation that is not suggestive of this tumor entity. Due to the overlap of clinical and radiological features with other types of renal tumors, histological confirmation is essential for any renal mass with atypical clinical or radiological presentation.