Paraganglioma of the urinary bladder - Can biologic potential be predicted?

BACKGROUND. Paraganglioma of the urinary bladder is rarely encountered and its biologic behavior is uncertain. The authors sought to determine the pro gnostic factors that would predict patient outcome. METHODS. The Mayo Clinic experience over 53 years with paraganglioma of the bladder was reviewed. All histologic slides from 16 patients were reviewed by the authors. Eight cases were examined immunohistochemically with cytok eratin (AE1/3, cytokeratin 7, and cytokeratin 20), vimentin, S-100 protein, neuroendocrine markers (chromogranin, synaptophysin, and neuron specific e nolase), p53 protein, and MIB-1. DNA ploidy was determined by digital image analysis in formalin fixed, paraffin embedded tissue. The mean follow-up w as 6.3 years (range, 0.4-16.4 years). RESULTS. Paraganglioma usually occurred in young adult women (mean age, 45 years; range, 16-74 years). The male-re-female ratio was 1 to 3. The common symptoms and signs were hypertension and hematuria. The rumors were usuall y located intramurally in the lateral and posterior wall of the bladder and were multifocal in 3 cases (18%). Seven patients were treated by transuret hral resection, eight by partial cystectomy, and one by radical cystectomy. T classification was T1 (1 patient), T2 (9 patients), T3 (2 patients), and T4b (4 patients). Ar the time of diagnosis, one patient had distant metast asis and one had regional lymph node metastasis. One patient developed meta stasis 1 year after diagnosis and died of the disease 1.5 years later. None of the patients with T1 or T2 tumors had recurrence or tumor progression. All tumors were aneuploid. The mean MIB-1 labeling index was 1.5% (range, 0 .03-7.0%). The tumor cells displayed immunoreactivity for S-100 protein and neuroendocrine markers and were negative for p53 (except 1 case) and cytok eratin. CONCLUSIONS. Paraganglioma of the urinary bladder occurs mostly in young ad ult women. Patients with tumor of advanced classification (greater than or equal to T3) are at risk of recurrence, metastasis, and dying of the diseas e, whereas patients in this study with T1 or T2 disease had favorable outco mes after complete tumor resection. (C) 2000 American Cancer Society.