Modern management and multidisciplinary care of bladder prostate rhabdomyosarcoma.
This review describes the shift in pediatric bladder/prostate rhabdomyosarcoma from radical upfront surgery toward fusion-informed risk stratification and multimodal, organ-sparing care coordinated by multidisciplinary surgical teams.
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This review describes the shift in pediatric bladder/prostate rhabdomyosarcoma from radical upfront surgery toward fusion-informed risk stratification and multimodal, organ-sparing care coordinated by multidisciplinary surgical teams.
Research significance
The review reports that contemporary chemotherapy, radiation, delayed surgery, and PAX-FOXO1–informed risk stratification are used to balance tumor control with bladder preservation; it further proposes, rather than directly demonstrates, that closer collaboration between pediatric urology and pediatric surgery may reduce protocol deviations, inadvertent upstaging, and operative burden.
Source abstract
PURPOSE OF REVIEW: Bladder/prostate rhabdomyosarcoma (BP-RMS) is a challenging clinical entity within pediatric genitourinary oncology. While historical management relied on radical extirpative surgery, the contemporary paradigm has shifted toward multimodal therapy combining chemotherapy, radiation, and organ-sparing surgery to maximize bladder preservation. This review examines the current landscape of BP-RMS diagnosis, molecular risk stratification, and surgical management, with a particular emphasis on multidisciplinary surgical collaboration. RECENT FINDINGS: Outcomes for BP-RMS have improved through cooperative group trials (COG, SIOP, EpSSG), though functional bladder outcomes remain suboptimal despite organ preservation. Risk stratification has evolved to prioritize PAX-FOXO1 fusion status over histology alone, as fusion-positive tumors demonstrate more aggressive behavior. Surgical management now favors biopsy followed by delayed primary excision or radiation rather than upfront resection. Collaboration between pediatric urology and pediatric surgery in the surgical management of patients with BP-RMS may aid in optimizing oncologic outcomes, minimize trial protocol deviations, inadvertent upstaging, and reduce cognitive load during complex resections. SUMMARY: BP-RMS requires nuanced management that balances oncologic control with long-term functional quality of life. The high complexity of pelvic surgery and the risks of inadvertent upstaging necessitate a cohesive, multidisciplinary surgical team to navigate the anatomical and therapeutic challenges of this disease.