Clear Cell Sarcoma of the Kidney: A case report

ABSTRACT

Clear Cell Sarcoma of the Kidney(CCSK) is a rare malignant childhood tumor which is distinguished from Wilms tumor by its pathologic features, clinical presentation and frequent occurrence of metastasis to bone. We report a case of CCSK from a 2 year-old girl in the right kidney, followed by metastasis to thoracic vertebrae and left temporal lobe. Histogenesis of this tumor is controversial, although some studies suggest primitive mesenchymal origin. This case was studied with the aids of immunohistochemistry and electron microscopy in an effort to verify the histogenesis of the tumor. Vimentin was reactive in tumor cell, but cytokeratin, GFAP, S-100 protein and desmin were not stained, which confirmed the previous reports by others. Ultrastructural observation of the tumor cells showed neither features of epithelial cell nor differentiated mesenchymal cells.