Papillary urothelial tumor of low malignant potential in a pediatric patient: case associated with Poland syndrome

Papillary urothelial tumor of low malignant potential is rare in pediatric patients, accounting for only 0.1-0.4 % of bladder tumors under 20 years of age. We present the case of a 14-year-old female with Poland syndrome and history of laryngeal, nasal, and colonic polyposis presented with intermittent gross hematuria, suprapubic pain, and urinary incontinence. Transurethral resection (TUR) identified a papillary urothelial neoplasm of low malignant potential. Genetic testing revealed an ARMC4 variant (primary ciliary dyskinesia), with no known association to PUNLMP. This case highlights the importance of a stepwise approach including early imaging, histologic confirmation, and endoscopic monitoring.