Pediatric Radiology

, Volume 39, Issue 7, pp 723–726 | Cite as

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

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

Neuroblastoma Opsoclonus-myoclonus syndrome Adrenal tumor Children 

References

  1. 1.
    Cushing H, Wolbach SB (1927) The transformation of a malignant paravertebral sympathicoblastoma into a benign ganglioneuroma. Am J Pathol 3:203–216PubMedGoogle Scholar
  2. 2.
    Rudnick E, Khakoo Y, Antunes NL et al (2001) Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: clinical outcome and antineuronal antibodies. A report from the Children’s Cancer Group Study. Med Pediatr Oncol 36:612–622PubMedCrossRefGoogle Scholar
  3. 3.
    Pranzatelli MR (1992) The neurobiology of the opsoclonus-myoclonus syndrome. Clin Neuropharmacol 15:186–228PubMedCrossRefGoogle Scholar
  4. 4.
    Pranzatelli MR (1996) The immunopharmacology of the opsoclonus-myoclonus syndrome. Clin Neuropharmacol 19:1–47PubMedCrossRefGoogle Scholar
  5. 5.
    Russo C, Cohn SL, Petruzzi MJ et al (1997) Long-term neurologic outcome in children with opsoclonus-myoclonus associated with neuroblastoma: a report from the Pediatric Oncology Group. Med Pediatr Oncol 29:284–288CrossRefGoogle Scholar
  6. 6.
    Fox F, Davidson J, Thomas LB (1959) Maturation of sympathicoblastoma into ganglioneuroblastoma: report of two patients with 20- and 46-year survivals respectively. Cancer 12:108–116PubMedCrossRefGoogle Scholar
  7. 7.
    Dargeon HW (1962) Neuroblastoma. J Pediatr 61:456–471PubMedCrossRefGoogle Scholar
  8. 8.
    Kinsbourne M (1962) Myoclonic encephalopathy of Infants. J Neurol Neurosurg Psychiatr 25:271CrossRefGoogle Scholar
  9. 9.
    Solomon GE, Chutorian AM (1968) Opsoclonus and occult neuroblastoma. N Engl J Med 279:475–477PubMedGoogle Scholar
  10. 10.
    Mitchell WG, Davalos-Gonzalez Y, Brumm VL et al (2002) Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae. Pediatrics 109:86–98PubMedCrossRefGoogle Scholar
  11. 11.
    Pranzatelli MR, Tate ED, Travelstead AL et al (2006) Rituximab (anti-CD20) adjunctive therapy for opsoclonus-myoclonus syndrome. J Pediatr Hematol Oncol 28:585–593PubMedCrossRefGoogle Scholar
  12. 12.
    Blaes F, Fuhlhuber V, Korfei M et al (2005) Surface-binding autoantibodies to cerebellar neurons in opsoclonus syndrome. Ann Neurol 58:313–317PubMedCrossRefGoogle Scholar
  13. 13.
    Baker ME, Kirks DE, Korobkin M et al (1985) The association of neuroblastoma and myoclonic encephalopathy: an imaging approach. Pediatr Radiol 15:184–190PubMedCrossRefGoogle Scholar
  14. 14.
    Parisi MT, Hattner RS, Matthay KK et al (1993) Optimized diagnostic strategy for neuroblastoma in opsoclonus-myoclonus. J Nucl Med 34:1922–1926PubMedGoogle Scholar
  15. 15.
    Swart JF, De Kraker J, Van Der Ley N (2002) Metaiodobenzylguanidine total-body scintigraphy required for revealing occult neuroblastoma in opsoclonus-myoclonus syndrome. Eur J Pediatr 161:255–258PubMedCrossRefGoogle Scholar
  16. 16.
    Tate ED, Allison TJ, Pranzatelli MR et al (2005) Neuroepidemiologic trends in 105 US cases of pediatric opsoclonus-myoclonus syndrome. J Pediatr Oncol Nurs 22:8–19PubMedCrossRefGoogle Scholar
  17. 17.
    No authors listed (1995) Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 27-1995. A 29-month-old girl with worsening ataxia, nystagmus, and subsequent opsoclonus and myoclonus. New Engl J Med 333:579–586Google Scholar
  18. 18.
    Hattner RS, Pounds TR, Matthay KK (1994) Normal cerebellar MIBG localization. Implications in the interpretation of delayed scans. Clin Nucl Med 19:985–988PubMedCrossRefGoogle Scholar
  19. 19.
    Gambini C, Conte M, Bernini G et al (2003) Neuroblastic tumors associated with opsoclonus-myoclonus syndrome: histological, immunohistochemical, and molecular features of 15 Italian cases. Virchows Arch 442:555–562PubMedGoogle Scholar

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

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