Poor outcome in patients with advanced stage neuroblastoma and coincident opsomyoclonus syndrome.
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.
Hiyama
E
E
Department of General Medicine, Hiroshima University School of Medicine, Japan.
Yokoyama
T
T
Ichikawa
T
T
Hiyama
K
K
Kobayashi
M
M
Tanaka
Y
Y
Ueda
K
K
Tanaka
Y
Y
Yano
H
H
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000235:genetics / Q000473:pathology; D002675:Child, Preschool; D004273:DNA, Neoplasm / Q000032:analysis; D017809:Fatal Outcome; D005260:Female; D005434:Flow Cytometry; D016259:Genes, myc; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000556:secondary; D009207:Myoclonus / Q000150:complications*; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000150:complications* / Q000235:genetics / Q000473:pathology; D015835:Ocular Motility Disorders / Q000150:complications*; D013577:Syndrome; D016896:Treatment Outcome
D004273:DNA, Neoplasm