Neuroblastoma with intraspinal (dumbbell) extension

Twenty-three patients with neurogenic tumors having extradural extension (dumbbell tumors) were treated over a 30-year period. Three had ganglioneuroma, 6 had ganglioneuroblastoma, and 14 had neuroblastoma. Nineteen patients with malignant tumors had symptoms of spinal-cord compression; three patients with ganglioneuroma and one with a malignant tumor had no neurologic symptoms. Delays in diagnosis were frequent ranging from 6 weeks to 3 years in 10 patients. Sixteen patients had abnormal x-rays of the spine. All patients with malignant tumors had positive myelograms. Three patients with ganglioneuroma and 13 with malignant tumors are alive and free of disease. Seven patients with malignant tumors died: five with stage IV and 1 with stage III tumors from disease, and 1 with stage II tumor during laminectomy. Age and stage were important prognostic factors; location was not. Treatment consisted of excision and radiation or radiation only. Patients receiving lower-dose (less than 2000 rad) radiation did as well as those receiving much larger doses. Morbidity in patients with malignant tumors was high; 4 have spinal deformity (3 severe) and 8 of 13 surviving patients have residual neurologic deficits. Earlier diagnosis, a surgical approach which does not introduce morbidity, and low-dose radiation in younger patients are important factors in reducing morbidity.