Clinical Characteristics, Recurrence, and Survival for Children and Young Adults With Urothelial Cancer: A Systematic Review.
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OBJECTIVE: To perform a systematic review of available data among children and young adults and propose a risk-based surveillance protocol. It was hypothesized that this population had favorable outcomes, with low rates of recurrence and death. METHODS: Medline was searched for peer-reviewed reports of pediatric and young adult (aged 21 years or below) urothelial cancer through July 2023. Demographic, clinical, and oncologic variables were extracted, with clinicopathological outcomes and survival described. RESULTS: Overall, 256 patients from 118 papers were included. There was a 2:1 male predominance, and the median age at diagnosis was 13 (IQR 10-16) years. 3.1% of patients had a known genetic syndrome and 78.5% presented with gross hematuria. 76% of patients had either papillary urothelial neoplasm of low malignant potential or low-grade urothelial carcinoma (UC), while 9% had high-grade UC. Regarding staging, 84% had non-muscle invasive and 5.1% had muscle invasive disease. Tumor recurrence was seen in 9% of patients with localized disease at presentation. Median time to recurrence after initial resection was 12 (IQR 6-21) months. The patients were followed for a median of 36 months (IQR 12-69). CONCLUSION: Children and young adult urothelial cancer patients have an excellent prognosis, but up to 10% can recur. The authors recommend long-term follow-up for these patients and herein suggest a risk-based protocol that balances the morbidity and costs of surveillance with the risk of recurrence and/or progression.