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

Abstract

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.

Localized dumbbell neuroblastoma: A study of 25 cases treated between 1982 and 1987 using the same protocol

“…Previous studies have reported similar results (7-15%) [1][2][3][4]. In our study, the frequency of dumbbell neuroblastomas is significantly higher in localized forms (23%) than in metastatic forms (8%) ( P = .0002), which confirms Punt's results [ 5 ] . The age of onset is under that of children presenting neuroblastomas without intraspinal extension; this notion has also been reported in other series [ 1,2,5].…”

“…Spinal decompression is a neurological emergency [9-111 and can be carried out by 3 methods: surgery, radiotherapy, or chemotherapy. Surgical decompression by laminectomy and excision of the intraspinal portion remains dogmatic for many [ 1, 5,12], permitting neurological improvement in 2/3 of cases [Id]. However complete neurological recovery is observed in only 30-40% of cases, [ 1 4 , 6 ] and seems to be better if neurological signs are more recent [2].…”

Malignant transformation of ganglioneuroma into spinal neuroblastoma in an adult

“…It has been suggested that spinal neuroblastoma in children may have a more favorable prognosis than this type of lesion in other locations, with a lower incidence of disseminated disease at presentation, especially in infants younger than 1 year of age. 12,13,17 The experience in adults is too limited to extrapolate this conclusion reliably, but, to our knowledge, no patient surviving more than 2 years has previously been reported. 1,9 Conclusions Ganglioneuroma, an otherwise benign tumor, appears to have the rare capacity to develop into neuroblastoma in a delayed fashion.…”

“…16 There is no consensus on the most appropriate therapy for these tumors in adults, but judging from the far greater pediatric experience, a combination of surgery, radiation, and chemotherapy would likely provide the optimum therapy. 13 Despite the availability of various therapeutic modalities, there have been no reports of survival beyond 2 years in an adult with this disease. 1,9 We present the first case of long-term survival in an adult with spinal neuroblastoma.…”

Late sequelae of symptomatic epidural compression in children with localized neuroblastoma

“…Neuroblastoma originates from cells of the primitive neural crest that eventually populate the sympathetic ganglia and the inner adrenal gland 1. In approximately one‐half of cases, the primary tumor arises at the level of the paravertebral ganglia and may infiltrate the adjacent intervertebral foramina and compress the intraspinal structures 2. Although modern imaging studies document the infiltration of the intervertebral foramina by the tumor in at least one‐third of neuroblastomas 3, only 5–7% of the cases develop symptoms related to epidural compression (EC) 4, 5.…”