Pediatric Radiology

, Volume 39, Issue 7, pp 723–726

The association between neuroblastoma and opsoclonus-myoclonus syndrome: a historical review

  • Alexis B. Rothenberg
  • Walter E. Berdon
  • Giulio J. D’Angio
  • Darrell J. Yamashiro
  • Robert A. Cowles
Historical Perspective

DOI: 10.1007/s00247-009-1282-x

Cite this article as:
Rothenberg, A.B., Berdon, W.E., D’Angio, G.J. et al. Pediatr Radiol (2009) 39: 723. doi:10.1007/s00247-009-1282-x

Abstract

An association between neuroblastoma and opsoclonus-myoclonus syndrome (OMS) was described as early as 1927 within the first report on the transformation of malignant neuroblastoma to a benign ganglioneuroma. It was not recognized at that time nor was it appreciated in the subsequent follow-up report on the same patient in 1959. Myoclonic encephalopathy of infancy, an alternative name for OMS, was described by a pediatric neurologist in 1962; however, its connection to neuroblastoma was not known. It was only in 1968 that the association between these two conditions was first reported. The neuroblastoma tumors associated with OMS are almost all small, stage I–II with no associated MYCN amplification or metastases. OMS occurs in 2–3% of patients with neuroblastoma, but neuroblastoma is found in as many as 50% of children who present with OMS. Nearly 100% of the children with neuroblastoma associated with OMS survive, and this has led to speculation that the OMS is a result of an autoimmune process, not metastases. Affected children are treated with steroids, ACTH, or intravenous immunoglobulin, but many have persistent neurologic and developmental deficits. Using the original case reported in 1927, we summarize a century of literature in this review on OMS and its association with neuroblastoma.

Keywords

NeuroblastomaOpsoclonus-myoclonus syndromeAdrenal tumorChildren

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Alexis B. Rothenberg
    • 1
  • Walter E. Berdon
    • 2
  • Giulio J. D’Angio
    • 3
  • Darrell J. Yamashiro
    • 4
  • Robert A. Cowles
    • 5
  1. 1.New York Medical CollegeValhallaUSA
  2. 2.Department of Radiology, Division of Pediatric RadiologyMorgan Stanley Children’s Hospital of New York-Presbyterian and Columbia University Medical CenterNew YorkUSA
  3. 3.Department of Radiation OncologyHospital of the University of PennsylvaniaPhiladelphiaUSA
  4. 4.Division of Pediatric OncologyMorgan Stanley Children’s Hospital of New York-Presbyterian and Columbia University Medical CenterNew YorkUSA
  5. 5.Department of Surgery, Division of Pediatric SurgeryMorgan Stanley Children’s Hospital of New York-Presbyterian and Columbia University Medical CenterNew YorkUSA