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CONGRESS OF THE ESPU
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S18-5 (PP)
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BLADDER EXSTROPHY AND THE RISK OF URINARY
BLADDER CANCER IN SWEDEN 1952-2012
Gisela REINFELDT ENGBERG, Magdalena FOSSUM and Agneta NORDENSKJÖLD
Astrid Lindgren Children's Hospital, Pediatric Surgery and Urology, Stockholm, SWEDEN
PURPOSE
Several reports suggest that exstrophied bladders have a significantly higher risk for developing
bladder cancer. The aim of this study was to evaluate the risk in a Swedish register study.
MATERIAL AND METHODS
A matched designed cohort study was performed by linkage-analysis of Swedish national registers
based on the total population born 1952–2012; the National cancer register, the Cause of death
register and the National patient registers were used. After ethical permission we included analyses
of medical records. Inclusion criteria were: born in Sweden with classical bladder exstrophy accord-
ing to ICD coding. We used 5 controls per patient matched for age and sex.
RESULTS
We identified 180 validated cases, 98 males and 82 females, with bladder exstrophy. Two males
and 2 females, were diagnosed with urinary bladder cancer comparing to none in controls. The
mean age at diagnose was 39 years. Hematuria was the main symptom and all had had cystoscopy
performed 1–4 years prior to diagnose. Two were classified according to tumor grading systems.
Both presented with grade T4, were surgically treated with cystectomy, developed metastasis within
a few weeks-months, and died within 13 months of first symptoms. In the age group of 35–61, in
total 76 cases, the risk of urinary bladder cancer was 5.3 %.
CONCLUSIONS
Exstrophy patients have an increased risk of developing bladder cancer at a young age. The
cancer seems aggressive. Cystoscopy had been performed in intervals prior to symptoms without
detection and only palliative treatment was an option. This warrants further attention for optimizing
surveillance programs.
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Discussion