Clinical presentation and outcome of high-grade urinary bladder leiomyosarcoma in adults

Objectives. To determine the clinical presentation and outcome of patients with high-grade bladder leiomyosarcoma. Methods. Between July 1986 and April 1998, 36 adult patients (mean follow-up 56 months) with a diagnosis of urinary bladder leiomyosarcoma were evaluated at the University of Texas M. D. Anderson Cancer Center. We retrospectively reviewed the records of these patients for information on clinical features, treatment, and outcome. Results. The mean age of the patients was 63 years. Twenty-six patients were white men in their seventh decade. The most common symptom at presentation was gross hematuria (81% of patients), followed by increased urinary frequency (28%), and dysuria (19%). Thirty-five patients were treated surgically for bladder leiomyosarcoma; of these, 12 (34%) developed recurrent disease (5 with local recurrence and 7 with distant metastasis), with a median time to diagnosis of recurrent disease of 8.3 months. The most common site of distant failure was the retroperitoneum. The disease-specific survival rate at 1, 3, and 5 years was 88.6%, 62.0%, and 62.0%, respectively. Multivariate analyses demonstrated that only the Memorial Sloan-Kettering Cancer Center disease stage system was a significant predictor of survival of patients with bladder leiomyosarcoma (P = 0.018). Conclusions. In patients with high-grade leiomyosarcoma of the bladder, radical cystectomy in the face of localized disease, whether at the initial presentation or after neoadjuvant chemotherapy, can result in a 5-year, disease-specific survival rate of 62.0%.