PARAURETHRAL GLAND CARCINOMA IN A MAN WITH BLADDER EXSTROPHY DIAGNOSED 41 YEARS AFTER BLADDER PLATE RESECTION

Abstract

The most frequent tumors of the lower urinary tract in patients with bladder exstrophy are adenocarcinoma, squamous cell carcinoma, transitional cell carcinoma and rhabdomyosarcoma of the bladder.1 We report a case of tubulopapillary paraurethral gland carcinoma that was diagnosed 41 years after bladder plate resection. CASE REPORT A 2-year-old boy with bladder exstrophy underwent bladder plate resection and ureterosigmoidostomy in 1956. In 1995 he was referred to our institution for recurrent pyelonephritis and potential conversion of the ureterosigmoidostomy into an orthotopic bladder substitute. During diagnostic evaluation flexible urethroscopy revealed a 10 mm. papillary tumor in the prosterior urethra at the level of the colliculus seminalis. Transurethral biopsy showed tubulopapillary tumor tissue with severe cellular atypia and a high mitotic rate with no lymph node or distant metastases. The patient underwent radical prostatovesiculectomy, posterior urethrectomy and conversion of the ureterosigmoidostomy into a Mainz II pouch in 1 surgical session. Histological analysis of the specimen revealed a soft friable tumor in the posterior urethra near the colliculus seminalis with exophytic growth. Microscopic analysis demonstrated tubulovillous invasive growing carcinoma with scant intracytoplasmic mucin, formation of a glycocalix and a high mitotic rate (see figure). Tumor cells were negative for prostate specific antigen (PSA), yet strongly positive for p53 tumor suppressor antigen (see figure). The postoperative course was uneventful. The patient is in excellent physical health and free of tumor recurrence 5 years after surgery. Serum creatinine and ultrasound of the kidneys are normal. By means of assisted reproduction he fathered twins in 1997 and, despite bladder plate resection and radical prostatectomy, is fully potent.