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Expression of the Neuronal Form of pp60^c-src in Neuroblastoma in Relation to Clinical Stage and Prognosis¹

Departments of Pathology, University of Uppsala, University Hospital [C. B., F. H., I. J., S. P.], and Pediatrics, University Hospital [F. H.], Uppsala, and Department of Clinical Genetics, Karolinska Hospital, Stockholm [M. N.], Sweden

The expression of the protooncogene c-src has been studied in specimens of childhood tumors with special reference to neuroblastoma and other tumors of neuronal origin. For comparison c-src gene expression was studied in seven neuroblastoma and neuroepithelioma cell lines. The structurally distinct neuronal product of the gene, pp60^c-srcN, expressed during normal development in neuroblasts and neurons, was identified by immunoblotting technique together with the fibroblast form, pp60^c-src. While pp60^c-src was found in most tumors studied, the neuronal form was restricted to neuroblastomas (23 of 27) and retinoblastomas (3 of 3) and could not be detected in the other childhood tumors. A dominance of the neuronal form, pp60^c-srcN, was exclusively found in the infant cases of neuroblastoma (9 of 12), estimated to have good prognosis. These results indicate that pp60^c-srcN might be a diagnostic marker in primitive childhood tumors. When expressed in higher amounts than pp60^c-src, pp60^c-srcN may be a positive prognostic marker in neuroblastoma, especially useful in the evaluation of infants. In addition, lack of pp60^c-srcN seems to be incompatible with low stage neuroblastoma.

¹ This work was supported by The Swedish Cancer Society, The Children Cancer Foundation of Sweden, HKH Kronprinsessan Lovisas förening för barnasjukvård, Hans von Kantzows, and Ollie och Elof Ericssons stiftelser.

² To whom requests for reprints should be addressed, at Department of Pathology, University Hospital, S-751 85 Uppsala, Sweden.

Received 4/30/90. Accepted 8/ 2/90.