Thesis (M.Sc.) - Cairo University - National Cancer Institute - Department of Pediatric Oncology

Pediatric clear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor accounting for 5% of pediatric renal tumors. It is bone metastasizing with poor prognosis. Aim of the work was to assess the incidence and treatment outcome of CCSK patients in children and to show patients{u2019} characteristics. Procedure: We included all patients diagnosed as CCSK in the period between January 2005 and June 2014 at National Cancer Institute, Egypt and Nasser Institute Hospital, Egypt 2Multicenter3. Patients{u2019} demographics, clinical presentation, pathology, and management were reviewed. Follow up was continued until June 2015. Results: Seventeen patients were identi{uFB01}ed in the de{uFB01}ned time interval, accounting for 6.2% of all renal tumors diagnosed at both hospitals. Median and mean age were 23 months & 30.7 months respectively. Abdominal swelling and hematuria were the most common presentations. Stages I, II, III& IV represented 7 cases (41%), 4 (23%), 5 (30%) & 1 (6%), respectively. All patients had upfront radical nephrectomy, followed by adjuvant chemotherapy. Abdominal radiotherapy was given for local stages II and III at a point of the treatment protocol. At time of final follow up; eleven patients (65%) were alive in CR and six patients (35%) died; three of them due to treatment related mortality (TRM), one patient due to disease progression and 2 patients relapsed before 1 year of end of therapy and died. Five- year EFS & OS were 47.8% & 68.6% respectively. Conclusions: pediatric CCSK is mainly a localized disease with a high tendency of local relapse and metastasis. The disease stage is the most important prognostic factors predictive of outcome (EFS)

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