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Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor. Only 4 cases of CCSK with vascular thrombus have been reported, and 2 of these were pediatric cases. One of the children had an intraatrial thrombus as well. We describe a 3-year-old boy who was diagnosed as having a Wilms tumor but did not respond to preresection chemotherapy. He underwent complete resection of the tumor under cardiopulmonary bypass. Histologic examination indicated that the tumor was a CCSK. The patient was then managed with appropriate chemotherapy and radiation therapy and is well 16 months after diagnosis.

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http://dx.doi.org/10.1016/j.jpedsurg.2011.09.050DOI Listing

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