Abstract：
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.
A single centre retrospective review was performed of all confirmed cases of CMN between October 2001 and January 2021.
Nine cases of CMN in patients under 12 months of age were identified. The histopathology, management and outcomes of these patients are discussed.
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.
A single centre retrospective review was performed of all confirmed cases of CMN between October 2001 and January 2021.
Nine cases of CMN in patients under 12 months of age were identified. The histopathology, management and outcomes of these patients are discussed.
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.
摘要：
背景：先天性中胚层肾瘤（CMN）是一种罕见的肾脏肿瘤，预后良好。曾经被认为是良性肿瘤，现在人们认识到它有复发和转移的风险,随后结局较差.遗传畸变如ETV6-NTRK3融合的潜力提高了在某些患者中靶向治疗的潜力。监测的最佳模式和频率尚不清楚。本研究旨在评估该机构在CMN方面的经验和长期结果。
方法：对2001年10月至2021年1月间所有CMN确诊病例进行单中心回顾性研究。
结果：在12月龄以下的患者中发现了9例CMN。组织病理学,讨论了这些患者的管理和结果。
结论：CMN总体预后非常好，但是确实存在一个结果不佳的子组。很难准确识别这一组靶向辅助治疗。
方法：对2001年10月至2021年1月间所有CMN确诊病例进行单中心回顾性研究。
结果：在12月龄以下的患者中发现了9例CMN。组织病理学,讨论了这些患者的管理和结果。
结论：CMN总体预后非常好，但是确实存在一个结果不佳的子组。很难准确识别这一组靶向辅助治疗。
