Abstract Background Congenital mesoblastic nephroma (CMN) is a rare tumour of the kidney with an overall excellent prognosis. Once considered a benign tumour, it is now recognized to carry a risk of recurrence and metastases with subsequent poor outcomes. The potential for genetic aberrations such as ETV6‐NTRK3 fusion raises the potential for targeted treatments in certain patients. The optimum mode and frequency of surveillance is unclear. This study aims to assess this institution's experience with CMN and long‐term outcomes. Methods A single centre retrospective review was performed of all confirmed cases of CMN between October 2001 and January 2021. Results Nine cases of CMN in patients under 12?months of age were identified. The histopathology, management and outcomes of these patients are discussed. Conclusion CMN overall has a very good prognosis, but a subgroup does exist that will have poor outcomes. It is difficult to accurately identify this group to target adjuvant therapy.
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