Primary Orthotopic Taeniamyectomized Sigmoid Neobladder after Radical Cystectomy in Pediatric Patients with Bladder/Prostate Rhabdomyosarcoma: 15 Years' Experience at a Single Center

Background: Here we present our experience of primary orthotopic taeniamyectomized sigmoid neobladder reconstruction following radical cystectomy/prostatocystectomy due to bladder/prostate rhabdomyosarcoma in children resistant to chemotherapy and/or radiotherapy.Methods: Of 52 patients diagnosed with persistent genitourinary rhabdomyosarcoma, 43 boys and six girls underwent radical prostatocystectomy or cystectomy with orthotopic taeniamyectomized sigmoid neobladder reconstruction between August 2003 and February 2019. Three patients did not undergo primary orthotopic neobladder reconstruction because of positive urethral margin. All patients were regularly followed-up, and oncological and functional data were recorded.Findings: All 52 patients were regularly followed-up, with an average follow-up duration of 48 months (range, 4-186 months). The final pathological diagnosis was embryonal rhabdomyosarcoma. Six boys died of recurrence/metastasis. The remaining 46 patients were tumor-free. Renal function was normal, with a mean estimated glomerular filtration rate of 114 ml/min/1·73 m2. The average reservoir capacity was 140 mL. Forty patients were followed-up for ≥12 months; 36 (90·0%, 33 boys and three girls) achieved daytime urinary continence, whereas only four did not achieve it.Interpretations: This is the first and largest case series of sigmoid neobladder reconstruction after radical cystectomy for pediatric patients with refractory bladder/prostate rhabdomyosarcoma. Primary orthotopic taeniamyectomized sigmoid neobladder is safe and technically feasible in pediatric patients. Moreover, primary neobladder reconstruction is reasonable for patients with low risk of fibrostenosis. This novel type of neobladder can offer satisfactory long-term functional outcomes and good quality of life with low morbidity.Funding Statement: This study was supported by the Natural Science Foundation of Guangdong Province, China (Grant NO.2015A030313291) and Guangdong Provincial Department of Education High-level University Construction Funding Southern Medical University Clinical Research Startup Program (LC2016PY030).Declaration of Interests: The authors declare no competing interests.Ethical Approval Statement: This study was approved by Medical Ethics Committee of the Zhujiang Hospital, Southern Medical University. Written informed consent (or assent) was obtained from all participants before the beginning of the study.