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

Cancer. 1994 Sep 15;74(6):1821-6. doi: 10.1002/1097-0142(19940915)74:6<1821::aid-cncr2820740627>3.0.co;2-a.


Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Gland Neoplasms / complications*
  • Adrenal Gland Neoplasms / genetics
  • Adrenal Gland Neoplasms / pathology
  • Child, Preschool
  • DNA, Neoplasm / analysis
  • Fatal Outcome
  • Female
  • Flow Cytometry
  • Genes, myc
  • Humans
  • Infant
  • Liver Neoplasms / secondary
  • Myoclonus / complications*
  • Neoplasm Staging
  • Neuroblastoma / complications*
  • Neuroblastoma / genetics
  • Neuroblastoma / pathology
  • Ocular Motility Disorders / complications*
  • Syndrome
  • Treatment Outcome


  • DNA, Neoplasm