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

Purpose: To report on the treatment of patients withnewly diagnosed neuroblastoma presenting with spinalcord compression (SCC).Patients and Methods: Of 1,462 children with neuroblastomaregistered between 1979 and 1998, 76 (5.2%)presented with signs/symptoms of SCC, including motordeficit in 75 patients (mild in 43, moderate in 22, severe[ie, paraplegia] in 10), pain in 47, sphincteric deficit in 30,and sensory loss in 11. Treatment of SCC consisted ofradiotherapy in 11 patients, laminectomy in 32, andchemotherapy in 33. Laminectomy was more frequentlyperformed in cases with favorable disease stages and inthose with severe motor deficit, whereas chemotherapywas preferred in patients with advanced disease.Results: Thirty-three patients achieved full neurologicrecovery, 14 improved, 22 remained stable, andeight worsened, including three who become paraplegic.None of the 10 patients with grade 3 motor deficit,eight of whom were treated by laminectomy, recoveredor improved. In the other 66 patients, the neurologicresponse to treatment was comparable for the threetherapeutic modalities. All 11 patients treated by radiotherapyand 26 of 32 patients treated by laminectomy,but only two of 33 treated by chemotherapy, receivedadditional therapy for SCC. Fifty-four of 76 patients arealive at time of the analysis, with follow-up of 4 to 209months (median, 139 months). Twenty-six (44%) of 54survivors have late sequelae, mainly scoliosis andsphincteric deficit.Conclusion: Radiotherapy, laminectomy, and chemotherapyshowed comparable ability to relieve orimprove SCC. However, patients treated with chemotherapyusually did not require additional therapy,whereas patients treated either with radiotherapy orlaminectomy commonly did. No patient presenting with(or developing) severe motor deficit recovered or improved.Sequelae were documented in 44% of survivingpatients.