Abstract
Opsoclonus-polymyoclonia syndrome (OPS) is one of the features of neuroblastoma. We experienced a case of neuroblastoma associated with OPS. This 3-year-old girl had complained of gait disturbance for eight months. At physical examination, a hard and large mass was palpable in the right upper abdomen, and she showed horizontal nystagmus, tremor of fingers, trunkal ataxia, and cerebellar ataxic gait. Laboratory data revealed elevation of serum LDH, NSE, and ferritin, and the urinary excretion of VMA and HVA was elevated. Both CT scan and MRI revealed a huge tumor in the right retroperitoneal space which invaded to the oposite side and involved superior mesenteric and bilateral renal arteries. Bone scan revealed no evidence of bone metastasis. Exploratory laparotomy and iliac bone marrow aspiration were performed, and pathological diagnosis was neuroblastoma (rosette-fibrillary type) stage IV B. N-myc analysis revealed single copy. After three courses of chemotherapy (CPM, THP-ADR, CDDP, and VP-16) , total excision of the tumor and intra-operative irradiation to the tumor bed (6MeV, 12Gy) were performed. After the operation, she has been still on post-operative chemothrapy, however all of the neurological symptoms have disappeared completely.
