ESPU Congress 2018 - Abstract Book

23 11–14 APRIL, 2018, HELSINKI, FINLAND S2: [Parallel session] CASE REPORTS Moderators: Serdar Tekgül (Turkey), Thomas Blanc (France) ESPU Meeting on Wednesday 11, April 2018, 14:00–15:00 14:00–14:02 S2-1 (CP) PRIMARY PRIMITIVE NEUROECTODERMAL TUMOUR (PNET) OF THE URINARY BLADDER: A RARE ENTITY Sadaf ABA UMER KODWAVWALA  1 , Bashir AHMED  1 , Sajid SULTAN  1 , Shamvil ASHRAF  1 , Muhammad MUBARAK  2 , Philip G RANSLEY  1 and Adeeb Ul Hasan RIZVI  1 1) Sindh Institute of Urology & Transplantation, Philip G. Ransley Department of Paediatric Urology, Karachi, PAKISTAN - 2) Sindh Institute of Urology & Transplantation, Department of Pathology, Karachi, PAKISTAN PURPOSE Primitive neuroectodermal tumours (PNET) that arise in the urinary bladder are an extremely rare occurrence. Very few cases have been reported so far in the literature. Here we present two cases of PNET of the urinary bladder. MATERIAL AND METHODS Two boys presented with lower urinary tract symptoms and one of them had terminal hematuria. Ultrasound revealed urinary bladder growth in both children. RESULTS Two boys, age 2 and 5.5 years respectively, having polypoid mass in the urinary bladder on ultra- sound underwent cystoscopy and CT scan to assess growth extent. In one it measured 4.6x4.7 cms hanging mass from posteriolateral wall of urinary bladder, and 5x4 cms mass hanging from anterior wall of the bladder in other. Biopsy performed endoscopically in one but was inconclusive. No local invasion or distant metastases were found on staging workup. Both underwent local excision of bladder growth with tumour free margins. Histopathology revealed round blue cell tumour in sub- mucosa without invasion of detrusor muscle. IHC marker showed positivity for CD-99 in both cases and diagnosis of PNET was established. Both received eight cycles of adjuvant chemotherapy CVD alternating with IE. Both of them are off treatment for 5.5 and 1.5 years respectively without any relapse and voiding symptoms. CONCLUSIONS Both clinician and pathologist must be aware of this rare entity during diagnosis and management of urinary bladder growth. Local surgical excision with adjuvant chemotherapy without radiotherapy is safe and effective for the management of this aggressive tumour.