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Case Reports
. 1988 Dec;133(3):596-608.

Blastemal cells of nephroblastomatosis complex share an onco-developmental antigen with embryonic kidney and Wilms' tumor. An immunohistochemical study on polysialic acid distribution

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Case Reports

Blastemal cells of nephroblastomatosis complex share an onco-developmental antigen with embryonic kidney and Wilms' tumor. An immunohistochemical study on polysialic acid distribution

J Roth et al. Am J Pathol. 1988 Dec.

Abstract

Previous investigations on polysialic acid of the neural cell adhesion molecule NCAM in human kidney have demonstrated its presence during nephrogenesis in embryonic kidney, absence in normal adult kidney, and reexpression in Wilms' tumor. These data showed that polysialic acid of NCAM is an onco-developmental antigen in human kidney and provided more direct evidence for the metanephric origin of Wilms' tumor. In the present study, five cases of Wilms' tumor associated with nephroblastomatosis complexes were immunohistochemically investigated with a monoclonal antibody for the presence of polysialic acid. Regardless of the type of nephroblastomatosis complex, ie, renal nodular blastema, simple tubular metanephric hamartoma, sclerosing metanephric hamartoma with adenoma, or incipient Wilms' tumor, immunoreactivity for polysialic acid was found in the blastemal cells, but was undetectable in all other structural elements. Because only blastemal cells exhibited a characteristic feature of embryonal differentiating metanephric derivatives, it appears that Wilms' tumor has its origin not exclusively in nodular renal blastema but rather in blastemal cells present in the various forms of nephroblastomatosis complex. The presence of polysialic acid of NCAM in blastemal cells in such lesions indicates that further events in addition to the expression of the embryonic form of this cell adhesion molecule may be involved in the pathogenesis of Wilms' tumor.

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