• Ekrem Ünal, Ebru Yilmaz, Alper Özcan, Bilgen Işik, Musa Karakükcü, Cüneyt Turan, Hülya Akgün, Figen Öztürk, Abdulhakim Coşkun, Mehmet Akif Özdemir, and Türkan Patiroğlu.
• Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Erciyes University, Kayseri, Turkey
• Turk J Med Sci. 2020 Feb 13; 50 (1): 18-24.

Background/AimNon-Wilms renal tumors (NWRTs) are rarely encountered in children. The aim of this study is to determine the treatment strategies, prognosis, outcomes, and survival of children with NWRTs at Erciyes University in Kayseri, Turkey.Materials And MethodsMedical records of all patients (n = 20) treated for NWRTs over a 23-year period (1995–2018) were reviewed retrospectively.ResultsThere was male predominance (female/male: 7/13); the median age at diagnosis was 3.2 years old (0.1–13.5 years old). The major histological groups included mesoblastic nephroma (MBN), (n: 5, 25%), malignant rhabdoid tumor (MRT), (n: 5, 25%), renal cell carcinoma, (n: 3, 15%), inflammatory myofibroblastic tumor (n: 2, 10%), multilocular cystic renal tumors (n: 2, 10%), metanephric adenoma (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 died because of progressive disease; the mortality rate was 30% (n: 6).ConclusionWe have made the first report of bilateral renal involvement of ES/PNET in the English medical literature. Physicians dealing with pediatric renal masses should be alert to the high mortality rate in children with MRT, MBN, and ES/PNET and they should design substantial management plans for NWRTs.This work is licensed under a Creative Commons Attribution 4.0 International License.

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