Introduction: Nephroblastoma is one of the most common solid tumours in children. The occurrence of extrarenal nephroblastoma is exceedingly rare. What can be defined as extrarenal Wilms' tumor must satisfy the following criteria: histologically confirmed nephroblastoma and extrarenal location.
Material and methods: Current data on extrarenal nephroblastoma based on a selective review of the literature.
Results: The retroperineal location is reported to be typical in males, whereas the inguinal region is believed to predominate in females. There are no characteristic manifestations of extrarenal nephroblastoma. The symptoms depend on the location and stage upon diagnosis. US, CT and MRI are used to detect tumours in the retroperiteneal space, inguinal, sacro-coccal and scrotal area. However, extrarenal nephroblastoma does not show characteristic radiological features.
Conclusions: In absence of typical clinical presentation or conclusive imaging tests, the diagnosis is based on histology after the resection of the tumor. The recommended management of the extrarenal nephroblastoma is similar to the treatment of intrarenal nephroblastoma.
Keywords: extrarenal location; nephroblastoma.