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

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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.

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Authors and Affiliations

  1. New York Medical College, Valhalla, NY, USA

    Alexis B. Rothenberg

  2. Department of Radiology, Division of Pediatric Radiology, Morgan Stanley Children’s Hospital of New York-Presbyterian and Columbia University Medical Center, New York, NY, USA

    Walter E. Berdon

  3. Department of Radiation Oncology, Hospital of the University of Pennsylvania, Philadelphia, PA, USA

    Giulio J. D’Angio

  4. Division of Pediatric Oncology, Morgan Stanley Children’s Hospital of New York-Presbyterian and Columbia University Medical Center, New York, NY, USA

    Darrell J. Yamashiro

  5. Department of Surgery, Division of Pediatric Surgery, Morgan Stanley Children’s Hospital of New York-Presbyterian and Columbia University Medical Center, New York, NY, 10032, USA

    Robert A. Cowles

Authors
  1. Alexis B. Rothenberg
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  2. Walter E. Berdon
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  3. Giulio J. D’Angio
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  4. Darrell J. Yamashiro
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  5. Robert A. Cowles
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Corresponding author

Correspondence to Robert A. Cowles.

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Rothenberg, A.B., Berdon, W.E., D’Angio, G.J. et al. The association between neuroblastoma and opsoclonus-myoclonus syndrome: a historical review. Pediatr Radiol 39, 723–726 (2009). https://doi.org/10.1007/s00247-009-1282-x

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  • DOI: https://doi.org/10.1007/s00247-009-1282-x

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