RESUMEN
Immunohistochemical study and flow cytometric DNA analysis were done on seven cases of clear cell sarcoma of the kidney (CCSK) to speculate its histogenesis and to access the diagnostic usefulness of these methods in the differential diagnosis of Wilm's tumor. Clinically, CCSK is a rare malignant renal tumor of children with a propensity to metastasize to bone. Arborizing vascular pattern surrounding the tumor cells which have clear cytoplasm is characteristic histologic finding. Immunohistochemically, only vimentin was diffusely demonstrated in the tumor cell membrane and cytoplasm. In flow cytometric DNA analysis, four cases showed diploidy and two cases near diploidy. CCSK is a separate disease entity with characteristic clinicopathologic, immunohistochemical and flow cytometric findings in distinction from Wilms' tumor. Considering the histologic and immunohistochemical findings, the possible histogenetic mechanism of CCSK seems to be in common with congenital mesoblastic nephroma (CMN), that is primitive mesenchymal cells which committed early stromagenic activity.
Asunto(s)
Preescolar , Femenino , Humanos , Lactante , Masculino , ADN de Neoplasias/análisis , Diagnóstico Diferencial , Citometría de Flujo , Inmunohistoquímica , Neoplasias Renales/diagnóstico , Neoplasias de Células Germinales y Embrionarias/diagnóstico , Tumor de Wilms/diagnóstico , Ploidias , Valor Predictivo de las PruebasRESUMEN
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.