Multidisciplinary Approach to Genitourinary Rhabdomyosarcoma Treatment Leads to Consensus at Every Stage of Care

When a child is diagnosed with rhabdomyosarcoma of the genitourinary tract, parents focus on one thing: eliminating the cancer. But they are often relieved to discover that care teams at Cincinnati Children’s prioritize organ and fertility preservation as part of the treatment plan, too. 

Rhabdomyosarcoma is the most common subtype of soft tissue sarcoma, with about 350 childhood and adolescent cases in the United States per year. Approximately one in four rhabdomyosarcomas originate in the genitourinary tissues, including the paratestis, bladder and prostate.  

Rhabdomyosarcoma care has evolved in the last two decades, says pediatric urologist Andrew Strine, MD, who serves on the multidisciplinary rhabdomyosarcoma team at Cincinnati Children’s. Historically, children with large masses always had upfront resection before chemotherapy or radiation therapy. Surgeons removed the bladder and surrounding organs (prostate in males, uterus in females) and other tissues while trying to achieve negative margin. Urinary diversion required a bag or neobladder, with bladder reconstruction occurring later if possible. Fertility preservation techniques were nonexistent back then, and fertility was often lost due to the surgery or effects of chemo or radiation therapy.  

Now, pediatric hospitals that treat a high volume of genitourinary rhabdomyosarcoma are more likely to offer chemotherapy with or without radiation first. If there is residual disease after chemo and/or radiation, the team considers surgery, sometimes followed by additional therapy if needed.  

“Some patients can avoid surgery with this strategy, but if the patient does need surgery, the tumor is smaller and more easily resectable than it would have been at diagnosis,” Strine says. “Reducing the tumor with chemo or radiation often allows us to remove just part of the bladder or leave the bladder intact and only remove the prostate.” 

Survival outcomes have remained consistent for decades and can be as high as 90% depending on the tumor’s location, size, stage and genetic profile. But morbidity has decreased significantly. For example, bladder preservation rates are about 70 to 80% long-term, compared to the previous rate of about 20%.

Urology as an Integral Part of the Care Team

Patients with rhabdomyosarcoma are initially seen at the Cincinnati Children’s Cancer Center, and pediatric oncologists pull in additional specialties as needed.  

“The care of patients with rhabdomyosarcoma is always multidisciplinary,” says pediatric oncologist Brian Turpin, DO. “It is gratifying as a cancer specialist to share with a family that no decision is unilateral. Our urology, radiation oncology, radiology, pathology and oncology experts reach a consensus on every step, from diagnosis to local control, consolidation, systemic therapy and beyond, through recovery from acute side effects to long-term function preservation.” 

Prioritizing Future Fertility

Turpin adds that upfront involvement from urologists with expertise in this condition is essential.  

“One of the most satisfying aspects of caring for children with genitourinary rhabdomyosarcoma is delivering a confident plan to a family, knowing that the investment from our urology colleagues will not be limited to a finite role, such as removing the primary mass,” Turpin says. “Our entire team shares their commitment to ensuring the best success for preserving form and function throughout the patient’s life.” 

All patients diagnosed with rhabdomyosarcoma require chemotherapy, which puts them at high risk for long-term fertility issues. A pediatric urologist speaks to families whose sons are facing gonadotoxic therapy and presents fertility preservation options, which may include sperm cryopreservation or testicular tissue preservation under an experimental protocol. Pediatric gynecologists speak to families of female patients about the possibility of ovarian preservation. Services are available through the Cincinnati Children’s Comprehensive Fertility Care and Preservation Program, a collaboration of subspecialists in gynecology, oncology, urology and pathology that was established in 2009.   

“Uptake is about 30 to 40%, but we have good data indicating the families appreciate having the option regardless of whether they choose it,” says Strine, co-director of the fertility program. “They take it as a sign we are optimistic about their child’s prognosis.”  

To learn more, contact Andrew.Strine@cchmc.org. 

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