Perivascular epithelioid cell neoplasms represent a group of uncommon mesenchymal tumours with as yet undiscovered benign counterpart. Although perivascular epithelioid cell neoplasms have been described arising in most organ systems as well as in soft tissue and bone, only a small number of perivascular epithelioid cell neoplasms have been reported in the bladder. To date, there is no agreed system for predicting the behaviour of these tumours.
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