ObjectivesTo review the published cases of leiomyosarcoma of the urinary bladder and to report two further cases.MethodsThe databases Pubmed and Hinari were searched using the keywords ‘bladder’, ‘leiomyosarcoma’ and ‘smooth muscle neoplasm’. The 14 articles identified were reviewed, and we present a further two cases.ResultsOf more than 100 cases reported, 77 were reviewed. There is a lack of consensus about the standard treatment, and little is known about the natural history and prognosis of the tumour, due to its very low incidence. These tumours occur in older adults of either sex and are characterised by an aggressive behaviour. There is usually an unfavourable outcome, with the lungs being the most common site of metastasis. The two further cases we report had a different presentation and outcome.ConclusionsBecause of the limited experience with this rare tumour, there are insufficient data to suggest the optimum management strategy and prognosis.

Urothelial bladder tumour in childhood is extremely rare, and almost all the reported cases have been low-grade tumours with a favourable outcome. Here we review 57 reports comprising 127 cases, and we report two new cases.