Bladder cancer in patients with spina bifida: a serious risk

After bladder augmentation (BA) using bowel segments, it is known that there is a risk for secondary malignancies. It remains unclear whether this also applies to spina bifida (SB) patients without BA. The aim of this study was to analyze the frequency of bladder cancer (BC) in SB at a single tertiary institution and assess the patients' oncologic outcome.

Patients with SB and BC treated from January 2016 until March 2020 were included and corresponding data were collected retrospectively. Endpoints were progression-free survival (PFS) and overall survival (OS).

Among 132 adult patients with SB, four with a median age of 34.5 years (IQR 31.5-36.8, range 31-37) had a BC. None of the patients had undergone BA. Most common symptoms included recurrent urinary tract infections (UTI) (75%) and hydronephrosis (75%). At the time of the diagnosis, tumors were locally advanced (≥ T3) and lymph-node positive in all cases. All patients underwent radical cystectomy with adjuvant chemotherapy in two out of four cases. Histology showed squamous cell carcinomas (SCC) or at least a squamous cell component in all patients. The median PFS was 5.9 months (IQR 5.1-124.5) and the median OS was 8.7 months (IQR 6.3-125.5).

SCC in SB can appear at a young age and is usually diagnosed in an advanced tumor stage with poor prognosis despite radical surgical resection. Patients with SB without BA with clinical symptoms, new onset of hematuria, and/or upper tract dilatation should receive a cystoscopy ± further imaging.