İlknur BANLI CESUR, Begül YAĞCI KÜPELİ, Zerrin ÖZÇELİK

Wilms tümöründe tedavi sonuçları

Amaç: Bu çalişmanın amacı Wilms tümörlü (WT) hastaların tedavi sonuçlarını değerlendirmektir.Gereç ve Yöntem: 2012-2017 yılları arasında WT tanılı 21 hastanın klinik kayıtları geriye dönük olarak incelendi. Yaş, uyruk, semptomlar, teşhis ve cerrahi methotlar, cerrahi komplikasyonlar, hastalığın evresi, neoadjuvan kemoterapi durumu, radyoterapi durumu, rekürren hastalığın varlığı, tümörün histopatolojik özellikleri ve hastaların sonuçları analiz edildi. Bulgular: Kız/erkek oranı 2/1olup, ortalama yaş 4.21±2.26 yıldı. En sık saptanan fizik muayene bulgusu abdominal kitle idi. Hastaların %90,5’ine radyolojik bulgular ve klinik muayene ile tanı konuldu. Hastaların tamamına total nefroüreterektomi uygulandı. Hastaların %81’inde saptanan histopatolojik alt grup klasik Wilms tümörü idi. Hastaların %6,8’inde tanı anında uzak metastaz olup en sık uzak metastaz yeri akciğerdi. Hastaların genel sağkalımı (OS) %85,7 idi ve ortalama sağkalım süresi 36 aydı(2 ay-62 ay). Tanı anında ileri ever hastalık olması, uzak metastaz varlığı ve difüz anaplastik histoloji ile sağkalım oranları belirgin olarak etkilenmekteydi.Sonuç: Literatürdeki verilere benzer şekilde, çalışmamızın bulguları Wilms tümöründe ileri evre, uzak metastaz ve yaygın anaplastik histolojinin sağkalım üzerindeki olumsuz etkilerini doğrulamıştır. Uygun tedaviye karar vermek ve prognozu daha kesin olarak belirleyebilmek için tümörün moleküler özelliklerini araştıran büyük ölçekli çalışmalara ihtiyaç vardır.

Anahtar Kelimeler:

wilms, treatment, outcome

Treatment results of Wilms tumour

Purpose: The purpose of this study was to evaluate the treatment results of the patients with Wilms tumour (WT).Materials and Methods: The clinical records of 21 children with WT treated between 2012 and 2017 were retrospectively reviewed. Age, nationality, symptoms, diagnostic and surgical methods, surgical complications, the stage of the disease, the status of neoadjuvant chemotherapy, radiotherapy status, presence of recurrent disease and histopathological characteristics of the tumour and outcome of the patients were analyzed.Results: The female/male ratio was 2/1 and the mean age was 4.21±2.26 years. The most common physical examination finding was abdominal mass. The diagnosis was made with clinical examination and radiological investigation in 90.5% of the patients. Total nephroureterectomy was performed in all patients. The most common histopathological subgroup was classical Wilms Tumour that was detected in 81 % of the patients. 6.8 % of the patients had distant metastasis at the time of the diagnosis. The most common distant metastasis site was lungs. Overall survival (OS) of the patients was 85.7% and mean survival time was 36 months (2 months - 62 months). Advanced stage at diagnosis, presence of distant metastasis and diffuse anaplastic histology were associated with worse OS rates. Conclusion: Similar to existing knowledge on outcome of patients with Wilms tumor, our results confirmed the negative effect of advanced stage, distant metastasis and diffuse anaplastic histology on survival. Further large-scale studies investigating molecular features of the tumor are needed to decide for proper treatment and to better predict outcome.

Keywords:

Wilms tumor, survival, children,

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