Taking a Collaborative Approach to Deliver Consensus-Driven Treatment at Every Stage
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%.
