Poor outcome in patients with advanced stage neuroblastoma and coincident opsomyoclonus syndrome.

Abstract

Most patients with neuroblastoma who present with opsomyoclonus have a good prognosis. Neuroblastomas from such patients have been reported to contain a single copy of the N-myc gene. The authors describe three cases of patients with advanced neuroblastoma with opsomyoclonus, which had poor outcomes despite multimodal therapy. Amplification and expression of the N-myc gene were examined in these three primary tumors using Southern and Northern blot analyses. Then, flow cytometric analysis of the cellular DNA contents of these tumors was performed. N-myc amplification was observed in two tumors and N-myc RNA overexpression was observed in all three. Analysis of the cellular DNA contents of the tissue specimens revealed hyperdiploidy in all three tumors; one had a triploid index and the other two had hypotetraploid indexes. These findings suggest that a poor outcome for patients with opsomyoclonus may be associated with N-myc gene activation (amplification and/or overexpression) and that a hyperdiploid tumor is not always associated with a good prognosis.