Alper ÖZCAN, Ekrem ÜNAL, Ebru YILMAZ, Abdulhakim COŞKUN, Figen ÖZTÜRK, Cüneyt TURAN, Musa KARAKÜKÇÜ, Türkan PATIROĞLU, Bilgen IŞIK, Hülya AKGÜN, Mehmet Akif ÖZDEMİR

Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

Background/aim: Non-Wilms renal tumors (NWRTs) are rarely encountered in children. The aim of this study is to determine thetreatment strategies, prognosis, outcomes, and survival of children with NWRTs at Erciyes University in Kayseri, Turkey.Materials and methods: Medical records of all patients (n = 20) treated for NWRTs over a 23-year period (1995–2018) were reviewedretrospectively.Results: There was male predominance (female/male: 7/13); the median age at diagnosis was 3.2 years old (0.1–13.5 years old). Themajor histological groups included mesoblastic nephroma (MBN), (n: 5, 25%), malignant rhabdoid tumor (MRT), (n: 5, 25%), renalcell carcinoma, (n: 3, 15%), inflammatory myofibroblastic tumor (n: 2, 10%), multilocular cystic renal tumors (n: 2, 10%), metanephricadenoma (n: 1, 5%), renal neuroblastoma (n: 1, 5%), and bilateral renal Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET)(n: 1, 5%). All of the patients with NWRTs had radical nephrectomy except the child with bilateral renal ES/PNET. Six children diedbecause of progressive disease; the mortality rate was 30% (n: 6).Conclusion: We have made the first report of bilateral renal involvement of ES/PNET in the English medical literature. Physiciansdealing with pediatric renal masses should be alert to the high mortality rate in children with MRT, MBN, and ES/PNET and theyshould design substantial management plans for NWRTs.

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