Abstract

Neuroblastoma is the most common extracranial solid tumor of childhood which presents various clinical symptoms depending on the primary and metastatic sites. However, it has been rarely reported that sudden onset of blindness was the chief complaint of neuroblastoma. A four years old boy was admitted to the Yeungnam University Hospital with the chief complaint of a sudden onset of blindness due to a distant metastasis of abdominal neuroblastoma to the sphenoid sinus. On admission, both side pupils were dilated without light reflex, fundoscopy showed pale optic disk, electroretinogram was subnormal and visual evoked potential showed no response. The liver was palpable in 3 ½ finger breadth from the right costal margin and adult fist sized mass was palpable in the right flank. Skull X-ray showed destructed sphenoid bone and clinoid process and brain CT scan showed tumor mass in the sphenoid sinus and left orbit. Ultrasonogram and CT scan of the abdomen showed large tumor masses around the right kidney and para-aortic and retropancreatic lymph node. IVP showed displaced right calyceal system with preserved contour. Left supraclavicular lymph node which appeared after admission was biopsied and it showed poorly differentiated neuroblasts. He was treated according to the multiagent chemotherapy schedule for stage IV neuroblastoma patient of children's cancer study group. Abdominal tumor masses and sphenoid sinus mass were markedly reduced after 2 courses of the combination chemotherapy of cyclophosphamide, vincristine, DTIC, adriamycin and VM-26. Eventhough the blindness was not improved, the patient has been in good clinical codition.