RHABDOID KIDNEY TUMORS IN CHILDREN: A REVIEW OF THERAPEUTIC EVIDENCE

Abstract

Renal rhabdoid tumors in children represent a rare and highly aggressive malignancy characterized by rapid clinical progression, high metastatic potential, and a poor prognosis, particularly in patients diagnosed during the first years of life. This study aimed to review the available scientific evidence regarding the main therapeutic strategies used to manage this neoplasm, highlighting recent advances, prognostic factors, and future treatment prospects. This is an integrative literature review conducted via searches in the PubMed/MEDLINE, Scopus, Web of Science, Embase, Virtual Health Library (VHL), SciELO, and Cochrane Library databases, using descriptors related to renal rhabdoid tumors, pediatric oncology, and therapeutic modalities. Studies published between 2015 and 2025 were included if available in full text and addressing clinical, surgical, chemotherapy, radiotherapy, and emerging therapy aspects regarding the treatment of this neoplasm. Analysis of the studies demonstrated that a multimodal approach—comprising radical nephrectomy combined with intensive chemotherapy and radiotherapy in selected cases—remains the primary therapeutic strategy, although survival rates remain limited due to the tumor's high aggressiveness and the frequent presence of metastases at diagnosis. Furthermore, advances in the understanding of molecular biology, particularly regarding *SMARCB1* gene inactivation, have driven the development of targeted therapies and precision medicine-based strategies; while these show promising results, they still require validation through clinical studies with greater methodological robustness. In conclusion, the treatment of renal rhabdoid tumors in children remains a significant challenge for pediatric oncology, underscoring the critical need for expanded multicenter research, the standardization of therapeutic protocols, and the development of new personalized approaches capable of improving patient survival and quality of life.