Pediatric Rhabdomyosarcomas of the Genitourinary Tract

Jennifer T. Castle, Brittany E. Levy, Derek B. Allison, David A. Rodeberg, Eric J. Rellinger

Research output: Contribution to journalReview articlepeer-review


Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric and adolescent population, with 350 new cases diagnosed each year. While they can develop anywhere in the body, the genitourinary tract is the second most common primary location for an RMS to develop. Overall survival has improved through the increased use of protocols and multidisciplinary approaches. However, the guidelines for management continue to change as systemic and radiation therapeutics advance. Given the relative rarity of this disease compared to other non-solid childhood malignancies, healthcare providers not directly managing RMS may not be familiar with their presentation and updated management. This review aims to provide foundational knowledge of the management of RMSs with an emphasis on specific management paradigms for those arising from the genitourinary tract. The genitourinary tract is the second most common location for an RMS to develop but varies greatly in symptomology and survival depending on the organ of origin. As the clinical understanding of these tumors advances, treatment paradigms have evolved. Herein, we describe the breadth of presentations for genitourinary RMSs with diagnostic and treatment management considerations, incorporating the most recently available guidelines and societal consensus recommendations.

Original languageEnglish
Article number2864
Issue number10
StatePublished - May 2023

Bibliographical note

Funding Information:
Jennifer T. Castle is funded by the NIH training grant T32CA160003. Eric J. Rellinger is funded through an NIH COBRE Grant P20GM121327.

Publisher Copyright:
© 2023 by the authors.


  • bladder and prostate rhabdomyosarcoma
  • female genitourinary rhabdomyosarcoma
  • genitourinary rhabdomyosarcoma
  • paratesticular rhabdomyosarcoma
  • pediatric soft tissue sarcomas

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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