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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http://dx.doi.org/10.1111/ans.18165 | DOI Listing |
Virchows Arch
January 2025
Histology Laboratory, Children's Health Ireland, Dublin, Ireland.
Paediatric renal tumours include a broad range of neoplasms which largely differ, but also overlap to a smaller extent, with adult kidney cancer. These include the embryonal tumour nephroblastoma, which accounts for the majority of cases of kidney cancer in the first decade of life and, despite boasting a cure in ~ 90% cases, still presents clinical challenges in a small proportion of cases. A variety of less common mesenchymal tumours, including the mostly indolent congenital mesoblastic nephroma, clear cell sarcoma of kidney which continues to be associated with poor outcomes for higher stage disease, and the typically lethal malignant rhabdoid tumour, form the bulk of the remaining presentations in the first decade.
View Article and Find Full Text PDFWorld J Urol
December 2024
Department of Nuclear Medicine, Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, Kongjiang Road 1665, Yangpu District, Shanghai, 200092, China.
Mod Pathol
November 2024
Department of Pathology, Stanford University School of Medicine, Stanford, California. Electronic address:
Cureus
October 2024
Department of Pathology, Armed Forces Hospital Southern Region, Khamis Mushait, SAU.
Congenital renal tumors are rare. In infancy, congenital mesoblastic nephroma is the most commonly reported renal tumor. It is recognized antenatally due to polyhydramnios and presents clinically as a palpable abdominal mass in the neonatal period.
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