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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.