Clear cell sarcoma: a dilemma on pathological staging and clinical management

Clear cell sarcoma of the kidney (CCSK) has been classified as high risk tumour in the previous UK and international Wilms tumor studies. The current Society of Paediatric Oncology (SIOP) trial, UK version, advocates chemotherapy including doxorubicin prior to nephrectomy. Pathological staging and histology of the resected tumor are then applied to determine the extent and intensity of the postoperative therapy. We describe an unusual case with a trilobed tumor and debate the appropriate postoperative management.