Authors
Andrew T Gabrielson, David F Grabski, Timothy B Lautz, Edward M Gong
Published in
Current opinion in pediatrics. Aug 24, 2026. Epub Aug 24, 2026.
Abstract
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.
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.
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.
PMID:
42635013
Bibliographic data and abstract were imported from PubMed on 24 Aug 2026.
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