CLEAR-CELL SARCOMA OF THE KIDNEY IN CHILDHOOD

Clear-cell sarcoma of the kidney is malignant childhood tumor distingu ished from Wilm's tumor by microscopic and ultrastructural morphologic features and its tendency to metastasize to bone. From January 1984 t o December 1993, 186 cases with primary renal tumor were diagnosed, tr eated and followed-up in our institution. Ten of them were clear-cell sarcomas (5.4%). There were 7 boys and 3 girls with median age of 3 ye ars ranging from 1 to 10 years. The diagnosis was established by histo pathological features. Staging was done on the basis of clinical-labor atory and histopathologic findings. Laboratory studies consisted of ab dominal ultrasonography, two-dimensional chest X-ray and isotopic bone scan in all patients. Thoracic, abdominal or both CT scans were done in selected patients. Stage distribution was 7 and 3 patients in stage s II and III, respectively. After surgical intervention, all patients were treated according to the poor risk protocol of NWTS-III. Three pa tients relapsed within a 7-10 month period. All relapses were observed in bone. Two and five-year event-free survival rates were 44%, 44% re spectively.