Title

Single copies of the N-myc oncogene in neuroblastomas from children presenting with the syndrome of opsoclonus-myoclonus

Author

Cohn SL; Salwen H; Herst CV; Maurer HS; Nieder ML; Morgan ER; Rosen ST

Address

Division of Hematology/Oncology, Children's Memorial Hospital, Chicago, IL 60614

Source

Cancer, 62(4):723-6 1988 Aug 15

Abstract

Patients with neuroblastoma who present with the syndrome of opsoclonus and myoclonus enjoy a remarkably good prognosis independent of their stage of disease or their age at diagnosis. The presence of N-myc amplification also has been found to be an independent prognostic factor in neuroblastoma. Patients with multicopy N-myc tumors have rapid tumor progression whereas those with single-copy tumors have a significantly better progression-free survival. The authors examined four primary, untreated neuroblastomas for the N-myc copy number from patients who presented with opsoclonus and myoclonus. All four tumors had single copies of N-myc, and all four patients are alive with no evidence of recurrent disease with 6+ to 54+ months' follow-up. This appears to be the only report of N-myc analysis in this group of children. It would be interesting to analyze more neuroblastomas from patients who present with opsoclonus and myoclonus to determine how many of these patients have single N-myc copy tumors.

Language

English

Unique Identifier

88282296

MESH Headings

Child, Preschool ; Follow-Up Studies ; Gene Amplification ; Human ; Infant ; Myoclonus CO/*GE/TH ; Neuroblastoma CO/*GE/TH ; Oncogenes * ; Prognosis ; Support, Non-U.S. Gov't ; Syndrome

Publication Type

JOURNAL  ARTICLE

ISSN

0008-543X

Country of Publication

UNITED STATES