Patients with spina bifida and bladder cancer: atypical presentation, advanced stage and poor survival

Purpose: Patients with neurogenic bladder dysfunction due to spina bifida have been reported to be at increased risk for bladder cancer. Recent publications suggest that bladder augmentation is also a significant risk factor. We reviewed our experience with treating patients with spina bifida and bladder cancer. Materials and Methods: Patients with spina bifida treated for bladder cancer between 1995 and 2005 were identified. Patient demographics, mode of bladder management, risk factors and presenting symptoms were recorded along with therapy, pathological findings and outcome. This patient cohort was combined with all prior known published studies for analysis. Results: Eight patients with a median age of 41 years were treated. Only 1 patient (13%) had undergone bladder augmentation. Locally advanced stage J3 or greater) or lymph node metastases were present in 88% of cases. Median survival was 6 months with only I patient alive with no evidence of recurrence at 20 months. A total of 11 prior published cases were identified and combined with this series. Transitional cell carcinoma was present in 58% of patients. Median survival was 6 months. Only 37% of patients had undergone bladder augmentation. Conclusions: Patients with spina bifida and bladder cancer present at a young age with variable tumor histology and advanced stage, and they have poor survival. Presenting symptoms are often atypical and bladder cancer should be a consideration in this patient population, even in young adults. Due to poor survival further study is warranted in this population to determine whether screening would be beneficial for earlier detection and improved outcomes.