Neuroblastoma with symptomatic spinal cord compression at diagnosis: Treatment and results with 76 cases

Purpose: To report on the treatment of patients with newly diagnosed neurob lastoma presenting with spinal cord compression (SCC). Patients and Methods: Of 1,462 children with neuroblastoma registered betwe en 1979 and 1998, 76 (5.2%) presented with signs/symptoms of SCC, including motor deficit in 75 patients (mild in 43, moderate in 22, severe [ie, para plegia] in 10), pain in 47, sphincteric deficit in 30, and sensory loss in 11. Treatment of SCC consisted of radiotherapy in 11 patients, laminectomy in 32, and chemotherapy in 33. Laminectomy was more frequently performed in eases with favorable disease stages and in those with severe motor deficit , whereas chemotherapy was preferred in patients with advanced disease. Results: Thirty-three patients achieved full neurologic recovery, 14 improv ed, 22 remained stable, and eight worsened, including three who become para plegic. None of the 10 patients with grade 3 motor deficit, eight of whom w ere treated by laminectomy, recovered or improved. In the other 66 patients , the neurologic response to treatment was comparable for the three therape utic modalities. All 11 patients treated by radiotherapy and 26 of 32 patie nts treated by laminectomy, but only two of 33 treated by chemotherapy, rec eived additional therapy for SCC. Fifty-four of 76 patients are alive at ti me of the analysis, with follow-up of 4 to 209 months (median, 139 months). Twenty-six (44%) of 54 survivors have late sequelae, mainly scoliosis and sphincteric deficit. Conclusion: Radiotherapy, laminectomy, and chemotherapy showed comparable a bility to relieve or improve SCC. However, patients treated with chemothera py usually did not require additional therapy, whereas patients treated eit her with radiotherapy or laminectomy commonly did. No patient presenting wi th (or developing) severe motor deficit recovered or improved. Sequelae wer e documented in 44% of surviving patients. J Clin Oncol 19:183-190. (C) 200 1 by American Society of Clinical Oncology.