Extra-renal Wilms' tumour--is it different?

Nephroblastomas (Wilms' tumour) are the most common solid malignancies in childhood, but extra-renal Wilms' tumours are rare entities, and the diagnosis is made only after surgical intervention. However, the embryogenesis and the exact mechanism of occurrence of primary ERWTs is controversial, thereby presenting a number of unanswered questions of great theoretical interests. We present three well-proven cases of extra-renal Wilms' tumour together with a review of the literature based on its histogenesis, its behaviour and its management policies.