Ossifying renal tumor of infancy: report of a case with positive WT-1 immunohistochemistry and high mitotic index and review of the literature

Ossifying renal tumor of infancy (ORTI) is a rare, benign pediatric tumor of the kidney. Since first reported by Chatten in 1980, 23 cases have been published. Previous authors have argued that ORTI might originate from nephrogenic rests, thereby sharing a pathogenic relationship with Wilms' tumor (WT). ORTI is characterized histologically by a population of polygonal osteoblast-like cells around an osteoid core and densely cellular component of blastemal-like or spindle cells. While the immunohistochemical profile of the cellular components has been reported, to the best of our knowledge, the status of WT1 expression has only been reported once, where it showed negative marking. Mitoses have been described only sporadically in this neoplasm. We report on a case of ORTI with positive WT1 immunohistochemical marking and numerous mitoses. This case highlights a possible pitfall for misdiagnosing ORTI as a WT and provides additional insight into its pathogenesis.