Wilms Tumor: Review of the Current Literature with 32 Years of Experience of a Single Center

INTRODUCTION: Wilms Tumor (WT) is the most common renal tumor of childhood, usually seen between the ages of 2-4 years. The survival rate is reported as 90% in non-metastatic cases and 75% in metastatic cases. In the study, it was aimed to review the current literature by presenting 32 years of WT experiences of a single center.
METHODS: The files of 70 patients who underwent WT surgery in our Pediatric Surgery clinic between January 1988 and December 2019 were retrospectively reviewed.
RESULTS: Sixty-two of the 70 cases were unilateral and 8 were bilateral. 33 (53.2%) of unilateral cases were female, 29 (46.7%) of them were male, 5 (62.5%) of bilateral cases were female and 3 (37.5%) of them were male. The mean age was determined as 33.6 months (8-62) in unilateral cases and 25.4 months (12-54) in bilateral cases. WAGR Syndrome was found in one case. 5 of the cases were Stage I, 22 were Stage II, 19 were Stage III, 16 were Stage IV and 8 were Stage V. The most common metastasis was detected in the lung at the rate of 56% (n=9/16). While anaplasia was judged histopathologically in 12 (17.1%) of the cases, recurrence was detected in 10 (14.2%) cases. One case died peroperatively and 13 patients died during follow-up. 8 (61.5%) of those who were lost were undesirable in histopathology including anaplasia. The survival rate in our series was determined as 91% in positive cases and 33% in unfavorable cases.

DISCUSSION AND CONCLUSION: Future efforts in the treatment of WT should be to create national and international integrated data screening indexes in order to increase
recovery, reduce chemotherapy toxicity and contribute to innovations in treatment, especially in high-risk cases, as in our series.

Wilms Tümörü: Tek Merkezin 32 Yıllık Deneyimleri ile Güncel Literatürün Gözden Geçirilmesi

GİRİŞ ve AMAÇ: Wilms tümörü (WT) çocukluk çağının en sık renal tümörü olup genellikle 2-4 yaş arasında görülmektedir. Asıl tedavisi cerrahi olan WT olgularında sağ kalım oranı metastatik olmayanlarda %90, metastatik olanlarda %75 olarak bildirilmektedir. Çalışmada, tek merkezin 32 yıllık WT deneyimlerinin sunulması ile güncel literatürün gözden geçirilmesi amaçlandı.
YÖNTEM ve GEREÇLER: Ocak 1988 - Aralık 2019 arasında çocuk cerrahisi kliniğimizde WT nedeniyle ameliyat edilen 70 olgunun dosyaları geri dönük olarak incelendi.
BULGULAR: Yetmiş olgunun 62’si tek, 8’i çift taraflı idi. Tek taraflı olguların 33’ü (%53,2) kız, 29’u (%46,7) erkek, çift taraflı olguların 5’i (%62,5) kız, 3’ü (%37,5) erkek idi. Tek taraflı olgularda ortalama yaş 33.6 ay (8-62), çift taraflı olgularda 25.4 ay (12-54) olarak belirlendi. Bir olguda WAGR Sendromu, bir olguda at nalı böbrek saptandı. Olguların 5’i Evre 1, 22’si Evre 2, 19’u Evre 3, 16’sı Evre 4 ve 8’i Evre 5 idi. En sık metastaz %56 (n = 9/16) oranında akciğerde saptandı. Cerrahi olarak tek taraflı olgulara nefroüreterektomi, çift taraflı olgulara nefron koruyucu cerrahi uygulandı. Olguların 12’sinde (%17,1) histopatolojik olarak anaplazi belirlenirken 10 (%14,2) olguda nüks saptandı. 1 olgu peroperatif, 13 olgu ise takipte kaybedildi. Kaybedilenlerin 8’i (%61,5) anaplazi içeren unfavorable histopatolojide idi. Serimizde sağ kalım oranı, favorable olgularda %91, unfavorable olgularda ise %33 olarak belirlendi.
TARTIŞMA ve SONUÇ: WT tedavisinde gelecekteki çabalar, bizim serimizde olduğu gibi özellikle yüksek riskli olgularda iyileşmeyi artırmak, kemoterapi toksisitesini azaltmak ve tedavide yeniliklere katkı sağlamak amacıyla ulusal ve uluslararası entegrasyonlu veri tarama indeksleri oluşturmak olmalıdır.