| Summary: | Aim: We aimed to review our experience of managing Wilms tumor in our center over a period of fifteen years with regards to disease characteristics, outcome and see how well we are doing as compared to rest of the world. Methods: All children admitted at King Abdullah Specialist Children's Hospital, Riyadh from Jan 2001 to Dec 2015 with the diagnosis of Wilms tumor were included. Out of 39 eligible patients, 36 were included for final analysis whose treatment and follow up details were available. Results: Median age at diagnosis was 3 years, median duration of follow up was 7.2 years and 44% were male. Two (6%) patients had underlying hereditary predisposition syndromes. Abdominal distension was the most common symptom in 35(97%) patients and hypertension in 12(33%). Ten (28%) patients had stage I disease, 2(6%) had stage II, 13 (36%) had stage III, 8(22%) had stage IV while 3(8%) patients presented with stage V disease. Lung was the commonest metastatic site. Thirty-one (86%) patients had upfront nephrectomy. Anaplasia was present in 5(14%) of nephrectomy specimens. One patient had positive LOH for chromosomes 1p and 16q. All 6(17%) patients died of disease progression. Five year OS and EFS was 83% and 74% respectively. Anaplasia showed strong association with risk of relapse (p 0.0003). Conclusions: OS and EFS are comparable with rest of the developing world but we need to adopt modern tumor genetics based risk stratification to match the survival figures reported from developed world.
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