A Case of Ganglioneuroblastoma Associated With Opsoclonus-Myoclonus-Ataxia Syndrome, Treated by Subtotal Resection and Oral High-Dose Dexamethasone Pulse Therapy

Abstract

A 1-year-old boy, who had been suffering from irregular saccades of the eyes, limb myoclonus after falling asleep, and gait ataxia, was referred to our hospital for further examinations and was diagnosed as having opsoclonus-myoclonus-ataxia syndrome (OMS). Computed tomography revealed a retroperitoneal tumor. Imaging findings showed difficulty and a high risk of complete resection; thus, we performed the subtotal resection of the tumor. Histopathological examinations revealed that the tumor is a ganglioneuroblastoma. He was subjected to oral high-dose dexamethasone pulse therapy after surgery and all the OMS symptoms disappeared after the third course. No adverse effects and relapses were observed during the 21 courses of the therapy; thus, we considered stopping the therapy. Even if it was impossible to perform a complete tumor resection, as in our patient, a subtotal resection and postoperative immunosuppressive therapy might be effective for OMS in neuroblastoma patients.