Effect of risk group on survival in non-Wilms' renal tumors in children

Year 2022, Volume: 47 Issue: 4, 1578 - 1583, 28.12.2022

Ayşe Özkan İbrahim Bayram Kamuran Tutuş Gülay Sezgin Seyda Erdogan Serhan Küpeli

https://doi.org/10.17826/cumj.1166854

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.

Keywords

Non-Wilms renal tumors, children, risk groups, survival

Çocuklarda Wilms tümörü dışındaki böbrek tümörlerinde risk grubunun sağkalıma etkisi

Abstract

Amaç: Bu çalışmanın amacı Wilms tümörü dışındaki böbrek tümörleri (WDBT'leri) olan risk grubunun hastalığın klinik özelliklerini, tedavi stratejilerini ve özellikle sağkalımı etkilerini değerlendirmektir.3323
Gereç ve Yöntem: Ocak 2012 ile Ocak 2022 tarihleri arasında takip WDBT tanısı ile takip ve tedavi edilen hastalar çalışmaya dahil edildi (n=29; 16 erkek ve 13 kız). Hastalar, histolojik tiplerine göre yüksek ve düşük risk grubu olarak kategorize edildi. Tüm hastaların dosya kayıtları geriye dönük olarak incelendi. Hastaların klinik özellikleri, uygulanan tedaviler ve tedavi sonuçları analiz edildi.
Bulgular: Yüksek risk grubundaki hastalar; berrak hücreli sarkom (BHS) (n=8, %27,6), malign rabdoid tümör (MRT) (n=3, %10,3), renal hücreli karsinom (RHK) (n=2, %6,9), Hodgkin-dışı lenfoma (HDL) (n=1, %3,4), primitif nöroektodermal tümör (PNET) (n=1, %4) iken, düşük risk grubundaki hastalar; konjenital mezoblastik nefroma (KMN) (n=9, %31), anjiomiyolipom (n=4, %3,8) ve hemanjiom (n=1, %3,4) olarak saptandı. WDBT’li 29 hastadan 5’i progresif hastalık nedeniyle kaybedildi; ölüm oranı %17 idi (n=5) ve kaybedilen tüm hastalar yüksek risk grubunda idi (3 hasta MRT, 1 hasta BHS, 1 hasta PNET). 5 yıllık sağkalım oranı yüksek risk grubu için %72, düşük risk grubu için %100 olarak saptandı.
Sonuç: Düşük riskli tümörler, mükemmel sonuçlarla ilişkilendirilirken, yüksek riskli tümörler değişken bir sonuca sahiptir. MRT, BHS ve PNET'li çocuklarda yüksek ölüm oranlarına karşı dikkatli olmalı ve WDBT'ler için multidisipliner tedavi planları tasarlanmalıdır.

Keywords

Wilms tümörü dışındaki böbrek tümörleri, çocuklar, risk grupları

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