Late sequelae of localized neuroblastoma presenting with epidural compression. A study of the Italian and French Neuroblastoma Groups

Abstract

10053 Background: Symptomatic epidural spinal cord compression (EC) occurs in 5–10% of neuroblastoma (NB) patients at presentation. Survival is excellent, but sequelae of EC can be severe. Severity of motor impairment at presentation correlates with probability of neurological recovery, and chemotherapy (CT) and neurosurgery (NS) have been shown to be equally effective in relieving EC. However, so far only few studies focused on late sequelae of EC, mainly scoliosis. Methods: We retrospectively reviewed a cohort of children with localized NB, presenting with EC and surviving more than 2 years from time of diagnosis, to report late sequelae and correlate them with clinical features and tumour’s characteristics at diagnosis, and treatment. Chi-squared test, Odd-ratios and 95% confidence intervals were applied for statistical analysis. P<0.05 was considered statistically significant. Results: 102 eligible children were identified in the Italian and French NB Registries, and for 98 adequate information was available. Symptoms of EC at diagnosis included motor deficit in 94 cases and sphyncteric deficits in 33. Initial treatment consisted of CT in 66 cases, NS in 29 and radiotherapy in 3. Overall treatment consisted of CT alone in 44 cases, NS and CT in 38, radiotherapy and CT ± NS in 16. After a median follow-up of 7.3 years, 57children (58.2%) had one or more sequelae. Motor deficit at follow-up correlated only with severity of motor deficit at diagnosis. Spine deformities, mostly scoliosis, correlated with severity of motor deficit at diagnosis and at follow-up, length of follow-up and treatment including NS or radiotherapy. Sphyncteric dysfunctions correlated with severity of motor deficit at follow-up and presence of either dysfunction at diagnosis. Conclusions: More than half of children with localized NB and symptomatic EC enrolled into this study had one or more sequelae. The severity of motor deficit at diagnosis was the major factor accounting for the development and severity of late sequelae. Neurosurgery and radiotherapy increase the risk of spine deformities, which can develop after more than 10 years from diagnosis. No significant financial relationships to disclose.