Turkish Neurosurgery 1999 , Vol 9 , Num 3-4
1İstanbul Social Security Hospital, Neurosurgery Service, İstanbul Turkey
2University of İstanbul, Faculty of Medicine, Institute of Pediatric Oncology, İstanbul, Turkey
3University of Kentucky Medical Center, Department of Surgery, Division of Neurosurgery, Lexington
Included in this study were 56 children with primary extracranial Neuroblastomas (NB) (n=44) and Ganglioneuroblastomas (GNB) (n=12) who were admitted and treated at two institutions between January 1982 and March 1997. Thirteen of the 56 patients had epidural spinal canal invasion and spinal cord compression due to extension of primary tumor (Dumbbell Neuroblastoma-DNB). Five of these cases (case no: 4, 5, 10, 11, 12) were treated at the University of İstanbul, Institute of Pediatric Oncology, Turkey. The time from diagnosis of the primary disease to diagnosis of spinal canal invasion ranged from O day to 40 months. Of the 13 patients with epidural spinal canal invasion,4 had GNB and 9 had NB. Spinal cord compression was the initial clinical finding in eight of the NB patients. The neurological symptoms and signs varied from progressive spastic paraparesis to complete paraplegia with or without sphincter disturbances. In one of the patients with paraplegia, the tumor had also invaded the left lumbosacral plexus. Two of the thirteen patients had a congenital tumor, that were diagnosed soon after birth. Seven of the 13 cases had laminectomy and epidural tumor removal with or without chemotherapy and/ or radiation therapy, and six cases treated by radiotherapy and/ or chemotherapy. Histological assessment in two (case no:8 and 13) patients revealed that the disease had matured to GN. Four of the 13 patients died, 2 were lost during follow-up, and 7 patients went into remission and were followed for 8-84 months. At the time the results were analyzed, 4 patients were asymptomatic and 3 were experiencing neurologic sequelae. In conclusion; we diagnosed DNB in 23.2% of our NB and GNB cases. Chemotherapy should be the treatment of choice for DNB, even in the cases that recur after successful chemotherapy and associated with neurological impairment. Laminectomy and tumor removal is recommended for patients whose neurological symptoms rapidly worsen of in spite of chemotherapy. Keywords : Chemotherapy, dumbbell tumor, ganglioneuroblastoma, neuroblastoma, laminectomy
Corresponding author : Erol Taşdemiroğlu