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Clear cell sarcoma of the kidney: patients' characteristics and improved outcome in developing countries
Wael Zekri1, Ahmad Samir Alfaar, Dina Yehia
1Pediatric Oncology Department, Children's Cancer Hospital Egypt, Cairo, Egypt.
Background:
Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor accounting for 5% of pediatric renal tumors with an incidence of 20 patients per year in the USA. It is bone metastasizing with poor prognosis. Our aim was to show characteristics of patients in relation to improved outcome in one of the developing countries.
Procedure:
We included all patients diagnosed as CCSK in the period between July 2007 and March 2012 at Children's Cancer Hospital, Egypt. Patients' demographics, clinical presentation, pathology, and management were reviewed. Follow up was continued until April 2013.
Results:
Twenty-five patients were identified in the defined time interval, accounting for 7% all renal tumors diagnosed at the hospital. Mean age was 36 months. Abdominal swelling and hematuria were the most common presentations. Stages I, II, III, IV, and V represented 9 (36%), 3 (12%), 8 (32%), 3 (12%), and 2 (8%), respectively. Twenty-four patients had radical nephrectomy either upfront or after neo-adjuvant chemotherapy. Surgery was followed by adjuvant chemotherapy. Abdominal radiotherapy was given for local stages II and III. Twenty-two patients reached complete remission, while one patient had stationary disease and two patients died due to progression and relapse. Overall survival was 88.5% and event-free survival was 87.8% at 45 months.
Conclusion:
Although previous studies indicate poor prognosis of CCSK, our experience shows that those patients can be treated using extensive chemotherapy combined with proper local control.
Insights
Clear cell sarcoma of the kidney (CCSK) in children can be effectively treated with chemotherapy and local control, demonstrating improved outcomes even in developing countries. This aggressive tumor shows high survival rates with appropriate management.
Area of Science:
- Pediatric Oncology
- Nephrology
- Cancer Research
Background:
- Clear cell sarcoma of the kidney (CCSK) is a rare, aggressive pediatric renal tumor with a high propensity for bone metastasis and poor prognosis.
- CCSK accounts for approximately 5% of all pediatric renal tumors in the USA, with limited data from developing nations.
Purpose of the Study:
- To investigate the characteristics and treatment outcomes of pediatric patients diagnosed with CCSK in Egypt, a developing country.
- To evaluate the effectiveness of current treatment protocols in improving patient survival and remission rates for CCSK.
Main Methods:
- A retrospective review of all pediatric patients diagnosed with CCSK between July 2007 and March 2012 at Children's Cancer Hospital, Egypt.
- Data collected included patient demographics, clinical presentation, pathological findings, treatment modalities (surgery, chemotherapy, radiotherapy), and follow-up until April 2013.
Main Results:
- Twenty-five CCSK cases were identified, representing 7% of renal tumors at the hospital. The mean age was 36 months, with abdominal swelling and hematuria as common symptoms.
- High rates of complete remission (22/25 patients) and survival (88.5% overall survival, 87.8% event-free survival at 45 months) were achieved.
- Treatment involved radical nephrectomy, followed by adjuvant chemotherapy, with abdominal radiotherapy used for localized stages II and III.
Conclusions:
- Despite its aggressive nature and poor prognosis reported in prior studies, CCSK in children can achieve favorable outcomes with a comprehensive treatment approach.
- Extensive chemotherapy combined with appropriate local control strategies, including surgery and radiotherapy, is crucial for successful management of CCSK.
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