Neuroblastoma: a review of 21 cases presenting with spinal cord compression

The records of 21 children with neuroblastoma presenting with spinal cord compression, encountered over 17 years, were reviewed. Thirteen patients (61%) survive, free of neuroblastoma, at intervals ranging from nine months to 192 months (median, 78 months) from the time of diagnosis. The explanation for this relatively high survival rate was sought in an analysis of the cases which took into account age, site, extent of disease, and histology. The most significant features to emerge were the unusually high proportion of children under 12 months of age at presentation (11 of 21) most of whom survive (9 of 11) and the low incidence (3 of 21) of detectable at the time of diagnosis. The absence of a paraspinal mass was an unfavourable prognostic features (1 of 6 survives) whilst if a paraspinal mass was present, its anatomical level did not influence survival. In particular, children with retroperitoneal tumors fared no worse (survival, 6 of 7) than those with primary tumors at other sites (survival, 6 of 8). Morbidity was high (6 of 13), principally in infants with spinal cord compression from birth. Survival was also related to the histologic maturity of the tumor, even in the presence of metastases. Recommendations for management are made.