JU INSIGHT Long-term Urological Outcomes in Pelvic Genitourinary Rhabdomyosarcoma: A 48-Year Single-center Experience

By: Hsin-Hsiao Scott Wang, MD, MPH, MBAn, Boston Children’s Hospital, Massachusetts; Tenny R. Zhang, MD, NewYork-Presbyterian/Weill Cornell, New York; Venkat M. Ramakrishnan, MD, PhD, Brigham and Women’s Hospital, Boston, Massachusetts; Marie-Therese Valovska, MD, Brigham and Women’s Hospital, Boston, Massachusetts; Alan B. Retik, MD*, Boston Children’s Hospital, Massachusetts; Richard S. Lee, MD, Boston Children’s Hospital, Massachusetts | Posted on: 27 Jun 2023

*Posthumous Acknowledgments: The authors would like to express their endless gratitude for the many decades of mentorship, support, and teaching from Dr Alan B. Retik (1932-2022), who over the course of his long career served as Urologist-in-Chief, Surgeon-in-Chief, and Emeritus Urologist-in-Chief at Boston Children’s Hospital. The immense impact Dr Retik had on his patients, trainees, and the field of urology cannot be overstated.

Wang H-HS, Zhang TR, Ramakrishnan VM, Valovska MT, Retik AB, Lee RS. Long-term urological outcomes in pelvic genitourinary rhabdomyosarcoma: a 48-year single-center experience. J Urol. 2023;209(6):1202-1209.

Study Need and Importance

Rhabdomyosarcoma (RMS) is a rare pediatric soft tissue sarcoma affecting the genitourinary (GU) system in 15%-20% of cases. Although multimodal therapy with chemotherapy, radiation, and surgery has improved survival rates, morbidity is high. Yet, there are few long-term data on urinary and sexual function and quality of life.

What We Found

From 1970-2018, 51 patients at our institution were treated for GU RMS of the bladder, prostate, pelvis, vagina, and uterus with a median follow-up of 21 years. Twenty-six patients (51%) underwent up-front radical surgery (eg, cystoprostatectomy, pelvic exenteration) with staged continence mechanism creation (n=17). These patients had higher rates of continence (96.2% vs 58.3%, P = .003) compared to the 12 who initially underwent organ-sparing surgery (eg, partial cystectomy); other outcomes were comparable between the 2 groups (see Table). One-third (n=4) of organ-spared patients required additional major corrective surgery. In survey results from the AUA Symptom Score, International Index of Erectile Function, and Female Sexual Function Index, urinary complaints were mild, but both male and female patients reported significant sexual dysfunction.

Table. Postoperative Outcomes in Patients Managed With Radical vs Organ-sparing Surgery

Complication	Radical (n=26) No. (%)	Organ sparing (n=12) No. (%)	P value
Urolithiasis	10 (38.5)	2 (16.7)	.18
Recurrent UTI or pyelonephritis	7 (26.9)	3 (25.0)	.9
Urinary incontinence	1 (3.8)	5 (41.7)	.003
Stricture	5 (19.2)	1 (8.3)	.39
Fistula	2 (7.7)	0 (0)	.32
Abbreviation: UTI, urinary tract infection.

Limitations

Although this work represents one of the largest RMS populations reported, our cohort is relatively small and limited to a single-center retrospective analysis. The group is also heterogeneous, encompassing multiple types of GU RMS. We were not able to present oncologic outcomes given limitations in data collection. Finally, survey results were patient reported and subject to bias.

Interpretation for Patient Care

Although current Children’s Oncology Group protocols emphasize multimodal therapy with bladder conservation in GU RMS, our institutional experience in up-front radical surgery has demonstrated comparable or even favorable urological outcomes. Radical surgery should remain an option for appropriately selected patients. Regardless of treatment strategy, nearly all patients reported very poor sexual function.