A Case of Neuroblastoma Associated With Opsoclonus-Myoclonus-Ataxia Syndrome

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  • Opsoclonus-myoclonus-ataxiaを契機に発見された神経芽腫の1例
  • 症例報告 Opsoclonus-myoclonus-ataxiaを契機に発見された神経芽腫の1例
  • ショウレイ ホウコク Opsoclonus myoclonus ataxia オ ケイキ ニ ハッケン サレタ シンケイ ガ シュ ノ 1レイ

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Abstract

We report a case of neuroblastoma associated with opsoclonus-myoclonus-ataxia (OMA) syndrome. At 20 months of age, a previously healthy girl developed gait disturbance, tremor and nystagmus. At 24 months of age, she was referred for treatment to Kansai Medical University. Serum level of NSE and urine levels of HVA and VMA were elevated. Abdominal ultrasound and CT showed a 4-cm mass in the right adrenal gland. Metastatic workup by bone scan, MIBG scintigraphy, bone marrow aspirates and biopsies, and head CT were negative. The adrenal tumor was removed. Histological examination confirmed neuroblastoma, unfavorable histology. No N-myc amplification with high Ha-ras or trk A was revealed. Three weeks after surgery, most of the neurological symptoms disappeared. Four weeks after surgery, 6 courses of James' chemotherapy were performed. She has been well with residual intermittent ataxia with no tumor recurrence In the review of the Japanese literature, 40 patients had been reported as having neuroblastoma associated with OMA. OMA is a rare paraneoplastic or paraviral neurological syndrome. In children, OMA is most commonly associated with neuroblastoma, occurring in 2-3% of all children with this tumor. It is defined as acute onset of rapid and chaotic eye movements, myoclonic jerking of the limbs and trunk, and ataxia. While children with OMA and neuroblastoma most often display favorable prognosis tumor characteristics and a high survival rate, many also experience devastating developmental delays and nervous system dysfunction.

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