Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient

Pediatr Blood Cancer. 2020 Aug;67(8):e28218. doi: 10.1002/pbc.28218. Epub 2020 May 30.


Opsoclonus myoclonus syndrome (OMS) is a rare neurological syndrome caused by a paraneoplastic autoimmune process that affects children with neuroblastic tumors. Treatment includes corticosteroids, intravenous gamma globulin (IVIG), rituximab, and other immunosuppressive therapies. Here, we describe a patient diagnosed with OMS associated with a localized inflammatory myofibroblastic tumor. The patient has no evidence of tumor recurrence following surgical resection with 8-month follow-up. The neurologic symptoms resolved with corticosteroids and IVIG. This case demonstrates that in children, neoplasms other than neuroblastoma may be associated with this paraneoplastic syndrome, and highlights the importance of evaluating patients with OMS for underlying malignancies.

Keywords: IVIG; anaplastic lymphoma kinase; inflammatory myofibroblastic tumor; opsoclonus myoclonus syndrome.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adrenal Cortex Hormones / administration & dosage
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Child, Preschool
  • Follow-Up Studies
  • Humans
  • Immunoglobulins, Intravenous / administration & dosage
  • Immunosuppressive Agents / administration & dosage
  • Male
  • Neoplasms, Muscle Tissue / pathology
  • Neoplasms, Muscle Tissue / therapy*
  • Opsoclonus-Myoclonus Syndrome / pathology
  • Opsoclonus-Myoclonus Syndrome / therapy*
  • Rituximab / administration & dosage


  • Adrenal Cortex Hormones
  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Rituximab