A rare pediatric sarcoma affecting the kidney. It is characterized by the presence of epithelioid or spindle cells forming cords or nests, separated by fibrovascular septa. It is associated with internal tandem duplications in the BCOR gene. It metastasizes to lung, bone, brain and soft tissue. The disease is MONDO_0005006 (clear cell sarcoma of kidney). Also known as: CCSK, childhood clear cell sarcoma of the kidney, childhood kidney clear cell sarcoma, childhood renal clear cell sarcoma, clear cell sarcoma - kidney, clear cell sarcoma of the kidney, kidney clear cell sarcoma, paediatric kidney clear cell sarcoma.