An extrarenal Wilms' tumour exhibiting an unusual histology is reported in the inguinal region of a 3 year old girl. The tumour presents a tubulopapillary pattern associated with foci of nephroblastomatosis, a morphologic variant not previously described in any of the extrarenal Wilms' tumour. The unusual histologic picture enhances the difficulty in histogenetic based precise diagnosis. This case represents the 8th EWT of the inguinal region.
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