Treatment of Spinal Cord Compression

Spinal cord compression is considered a medical emergency. Patients receive immediate treatment because neurological recovery is more likely when symptoms are present for a relatively short time before diagnosis and treatment. Recovery also depends on the severity of neurological defects (weakness vs. paralysis). Neurological outcome appears to be similar whether cord compression is treated with chemotherapy, radiation therapy, or surgery, although radiation therapy is used less frequently than in the past.

The completed COG neuroblastoma clinical trials recommended immediate chemotherapy for cord compression in low-risk or intermediate-risk patients.[22,24,25] In a single study in this setting looking at the effect of glucocorticoids on neurological outcome, treatment was associated with improved early symptom relief. However, glucocorticoids did not prevent late residual impairment.[25]

Children with severe spinal cord compression that does not promptly improve or those with worsening symptoms may benefit from neurosurgical intervention. Laminectomy may result in later kyphoscoliosis and may not eliminate the need for chemotherapy.[22,24,25] Osteoplastic laminotomy, a procedure that does not remove bone, was thought to lessen spinal deformity. Osteoplastic laminotomy may be associated with a lower incidence of progressive spinal deformity requiring fusion, but there is no evidence that functional neurological deficit is improved with laminoplasty.[26]

The burden of long-term health problems in survivors of neuroblastoma with intraspinal extension is high. In a systematic review of 28 studies of treatment and outcome of patients with intraspinal extension, the severity of the symptoms at diagnosis and the treatment modalities were most associated with the presence of long-term health problems. In particular, the severity of neurological motor deficits was most likely to predict neurological outcome.[27] The severity of motor deficits at diagnosis is associated with spinal deformity and sphincter dysfunction at the end of follow-up, while sphincter dysfunction at diagnosis was correlated with long-term sphincter problems.[28] This supports the initiation of treatment before symptoms have deteriorated to complete loss of neurological function.

In a series of 34 infants with symptomatic epidural spinal cord compression, both surgery and chemotherapy provided unsatisfactory results once paraplegia had been established. The frequency of grade 3 motor deficits and bowel dysfunction increased with a longer symptom duration interval. Most infants with symptomatic epidural spinal cord compression developed sequelae, which were severe in about one-half of patients.[29]

An analysis of patients with intermediate-risk disease treated in the COG ANBL0531 [NCT00499616] study included 92 patients with intraspinal disease.[30] Of these patients, 42 (46%) were symptomatic. Among patients who were symptomatic, motor symptoms and bowel/bladder symptoms resolved completely in 73% and 88% of patients, respectively. Laminectomy or laminoplasty was performed in 22 of 42 symptomatic patients and was not significantly associated with improvements in symptoms.