Abstract
Background/aim Non-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 methods Medical records of all patients (n = 20) treated for NWRTs over a 23-year period (1995–2018) were reviewed retrospectively.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). 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). Conclusion We 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.
Highlights
Wilms tumor is the most common pediatric renal tumor and accounts for approximately 6%–7% of all pediatric malignancies [1]
We have made the first report of bilateral renal involvement of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) in the English medical literature
Physicians dealing with pediatric renal masses should be alert to the high mortality rate in children with malignant rhabdoid tumor (MRT), mesoblastic nephroma (MBN), and ES/PNET and they should design substantial management plans for non-Wilms renal tumors (NWRTs)
Summary
Wilms tumor is the most common pediatric renal tumor and accounts for approximately 6%–7% of all pediatric malignancies [1]. Pediatric non-Wilms renal tumors (NWRTs) constitute less than 10% of all renal tumors and have significantly higher mortality rates compared to childhood Wilms tumors [2,3,4,5,6,7,8]. There are controversies about the optimal follow-up and treatment plans for this rare and heterogeneous group of tumors. While the majority of cystic tumors have good prognosis, clinicians should be aware of aggressive tumors such as malignant rhabdoid tumors. NWRTs include mesoblastic nephroma, malignant rhabdoid tumor, renal cell carcinoma, inflammatory myofibroblastic tumor, multilocular cystic renal tumors, and metanephric
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