Bladder urothelial neoplasms in pediatric age: experience at three tertiary centers
A Berrettini1, M Castagnetti2, A Salerno3
1Department of Pediatric Urology, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Via Commenda 10, 20122 Milano, Italy.
Insights
Urothelial bladder neoplasms (UBN) are rare in children. This study found no recurrence or progression in young patients after transurethral resection of bladder tumor (TURB), suggesting less intensive follow-up may be appropriate.
Area of Science:
- Pediatric Urology
- Oncology
- Pathology
Background:
- Urothelial bladder neoplasms (UBN) are rare in pediatric patients, typically affecting older adults.
- Management and follow-up guidelines for pediatric UBN are lacking, leading to varied clinical practices.
- Gross painless hematuria is the most common presenting symptom in children with UBN.
Purpose of the Study:
- To investigate the outcomes of pediatric patients diagnosed with urothelial bladder neoplasms.
- To evaluate the recurrence and progression rates of UBN in patients under 18 years old.
Main Methods:
- Retrospective review of pediatric UBN cases treated in three tertiary pediatric urology centers (1999-2013).
- Classification of lesions using 2004 WHO/ISUP criteria: urothelial papillomas (UP), papillary urothelial neoplasm of low malignant potential (PUNLMP), low-grade urothelial carcinoma (LGUC), and high-grade urothelial carcinoma (HGUC).
- Analysis of treatment and follow-up protocols, including transurethral resection of bladder tumor (TURB), ultrasound (US), urine cytology, and cystoscopies.
Main Results:
- No disease recurrence or progression was observed in any patient after a median follow-up of 5 years.
- Lesions were predominantly solitary, non-muscle invasive, and low-grade (UP and PUNLMP).
- Management and follow-up strategies, including the use of TURB and surveillance methods, varied across the participating centers.
Conclusions:
- Urothelial bladder neoplasms are uncommon in children and adolescents, with a generally favorable outcome.
- Transurethral resection of the bladder tumor (TURB) is often effective for complete resection of pediatric UBN.
- A follow-up protocol primarily based on serial ultrasounds may be sufficient for pediatric patients, given the low recurrence and progression rates observed.
Introduction:
Urothelial bladder neoplasms (UBN) typically occur in patients in their sixth or seventh decade of life while they are infrequent in children and young adults. They occur in 0.1-0.4% of the population in the first two decades of life. Their management is controversial and paediatric guidelines are currently unavailable.
Objective:
To further expound the available data on the outcome of patients younger than 18 year old diagnosed with UBN.
Study Design:
We retrospectively reviewed the files of all the consecutive paediatric patients with UBN treated in three tertiary paediatric urology units from January 1999 to July 2013. Lesions were classified according to the 2004 WHO/ISUP criteria as urothelial papillomas (UP), papillary urothelial neoplasm of low malignant potential (PUNLMP), low-grade urothelial carcinoma (LGUC), and high-grade urothelial carcinoma (HGUC).
Results:
The table shows the results. Management after TURB varied among centres. One centre recommended only follow-up US at increasing intervals whereas another follow-up US plus urine cytologies and endoscopies, every three months in the first year, and at increasing intervals thereafter. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed disease recurrence or progression.
Discussion:
UBN is an uncommon condition in children and adolescents and, unlike in adults, its incidence, follow-up and outcome still controversial. Paediatric guidelines are currently lacking and management varies among centres. Gross painless haematuria is the most common presenting symptom. Therefore, this symptom should never be underestimated. US is generally the first investigation and additional imaging seems unnecessary. TURB often allows for complete resection. Lesions are generally solitary, non-muscle invasive, and low-grade (mainly UP and PUNLMP). Ideal follow-up protocol is the most controversial point. Reportedly, recurrence or progression during follow-up is uncommon in patients under 20 years, recurrence rate 7% and a single case of progression reported so far. Accordingly, a follow-up mainly based on serial US might be considered in this age group compared to adults where also serial endoscopies and urine cytologies are recommended. In the selection of the follow-up investigations, it should also be taken into consideration that urine cytology has a low sensibility in the detection of low-grade lesions while cystoscopy in young patients requires a general anaesthesia and hospitalization, and carries an increased risk of urethral manipulation.
Conclusion:
UBN is a rare condition in children. Ultrasound is generally accurate in order to visualize the lesion, and TURB can treat the condition. Lesions are generally low-grade and non-muscle invasive, but high-grade lesions can also be detected. In present series, after TURB, follow-up US monitoring at increasing intervals was used at all centres, follow-up cystoscopies were added in two centres, but with different schedules. Urine cytologies were considered only at one centre. After a median follow-up of 5 years (range 9 months-14.5 years), none of the patients showed recurrence or progression of the disease.
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