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Wilms' tumor generally presents as a painless abdominal lump in children, often in the flanks. Other causes of a painless abdominal lump are neuroblastoma, paraganglioma, lymphoma, rhabdomyosarcoma and desmoplastic small round cell tumor.

On histopathology, Wilms' tumor shows small cells with basophilic nuclei with an increased nucleus to cytoplasm ratio.