Affiliation:
1. Pediatrik Onkoloji Bilim Dalı ve Pediatrik Kemik İliği Transplantasyon Ünitesi
2. ÇUKUROVA ÜNİVERSİTESİ, TIP FAKÜLTESİ
Abstract
Purpose: The aim of this study was to evaluate tne effect of the risk group on the clinical features of the disease, treatment strategies and especially survival in children with Non-Wilms’ renal tumors (NWRTs).
Materials and Methods: Patients diagnosed with NWRTs followed up and treated between January 2012 and January 2022 were included in the study (n=29; 16 boys and 13 girls). They were categorized into high- and low-risk groups based on their histological type. Patient records were reviewed retrospectively. Clinical characteristics, treatments, and outcomes of these patients were analyzed.
Results: The high-risk group comprised clear cell sarcoma (CCSK) (n=8, 27.6%), malignant rhabdoid tumor (MRT) (n=3, 10.3%), renal cell carcinoma (n=2, 6.9%), non-Hodgkin lymphoma (n=1, 3.4%), and primitive neuroectodermal tumor (PNET) (n=1, 4%). The low-risk group comprised congenital mesoblastic nephroma (CMN) (n=9, 31%), angiomyolipoma (n=4, 3.8%), and hemangioma (n=1, 3.4%). Five patients died due to disease progression (mortality rate, 17%), all of whom belonged to the high-risk group (three patients with MRT, one with CCSK, one with PNET). The 5-year survival rate was 72% for the high risk group and 100% for the low risk group.
Conclusion: Although low-risk tumors are associated with excellent outcomes, the outcomes vary in high-risk tumors. Physicians should be wary of high mortality rates in children with MRT, CCSK, and PNET, and should design multidisciplinary treatments for NWRTs.
Subject
General Earth and Planetary Sciences,General Environmental Science
Reference11 articles.
1. Referans1 Fernandez CV, Geller JI, Ehrlich PF, van den Heuvel-Eibrink, Graf N, Mullen E et al. Renal tumors. In Pizzo and Poplack’s Pediatric Oncology, 8th ed. (Eds Blaney SM, Helman LJ, Adamson PC). Philadelphia: Wolters Kluwer, 2021;673-92.
2. Referrans2 Ahmed HU, Arya M, Levitt G, Duffy PG, Mushtaq I, Sebire NJ. Part I: Primary malignant non-Wilms’ renal tumours in children. Lancet Oncol 2007;8:730-7.
3. Referans3 Broecker B. Non-Wilms renal tumors in children. Urol Clin North Am 2000; 27: 463–9.
4. Referans4 Qureshi SS, Bhagat M, Verma K, Yadav S, Prasad M, Vora T et al. Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution. J Pediatr Urol 2020;16(4):475.e1-475.e9.
5. Referans5 van den Heuvel-Eibrink MM, Grundy P, Graf N, Pritchard-Jones K, Bergeron C, Patte C et al. Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: a collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups. Pediatr Blood Cancer 2008;50:1130–4.