Intraspinal neuroblastoma: Treatment options and neurological outcome of spinal cord compression

Abstract

Malignant spinal cord compression (MSCC) is a common complication of cancer. Paraspinal neuroblastoma (NB) in the thoracic, abdominal and pelvic regions may extend into the neural foramina causing compression of nerve roots and even the spinal cord. The prompt initiation of specific treatment can improve the neurological outcome. The aim of the present study was to review the clinical features, the management received and the factors that may affect the outcome of patients with MSCC caused by paraspinal NB. During a period between July 2007 and December 2012, a total of 576 NB patients were treated at the Children’s Cancer Hospital (Cairo, Egypt). Intraspinal disease extension was present in 51 patients (9%). The children with intraspinal disease extension were reviewed for disease pattern, neurological manifestations and treatment outcome. Children with intraspinal disease extension had an equal male to female ratio (1:1), and approximately two-thirds of patients (34/51) had a clinically manifested cord compression. The duration of neurological manifestations was >4 weeks in 58.8% (20/34) of symptomatic patients and ≤4 weeks in 41.2% (14/34). Subsequent to starting treatment, neurological manifestations showed a complete recovery in 16 patients (47.1%), partial in 11 (32.4%), and stationary course was found in 7 (20.6%). Manifestations of ≤4 weeks in duration carried an improved outcome compared with longer time compression, with a complete recovery in 78.6%, versus 25% for patients with a longer symptom duration (P=0.008). The upfront treatment, patient age and site of the primary tumor did not significantly affect the neurological outcome. Spinal cord compression in NB can be effectively managed with upfront chemotherapy. Initial surgical decompression should be reserved for benign variants only, including ganglioneuroma. Neurological manifestations of <4 weeks duration upon presentation are usually reversible.

Treatment and outcome of neuroblastoma with intraspinal extension: A systematic review

“…However, multivariate analysis indicated a significant association between motor sequela and neurosurgery . The evidence on the role of the symptom–diagnosis interval in the occurrence of late motor deficit is ambiguous; three studies report a significant better outcome with shorter symptom–diagnosis interval, while two studies found no association between the two parameters …”

“…To determine the outcome of this asymptomatic patient group is not straightforward, since it is often not assessed and/or reported . In three studies, it is explicitly mentioned that the asymptomatic patients do not have any long‐term health problems.…”

“…None of the studies reported that the outcome was assessed by an investigator who was blinded for the treatment status of the patients. Eight studies (28.6%) assessed the influence of prognostic/risk factors on survival, neurological outcome, and/or long‐term health problems . Only two studies (7.1%) adjusted for possible confounders in a multivariate analysis …”

“…Besides, treatment‐related spinal deformity is accompanied by tumor‐induced destruction of spinal anatomy making elucidation of each relative role hard, if not impossible. Three of the more recent studies found no significant difference between chemotherapy and neurosurgery in the ability to relieve neurological motor deficit . Due to small sample sizes and consequent limited statistical power, differences found were often not significant.…”

Overview and recent advances in the treatment of neuroblastoma

“…Late effects may also be related to the primary site of disease. For example, patients with spinal cord compression may have neurologic sequelae including paraplegia or sensory deficits, or alternatively may have significant scoliosis or kyphosis as a result of radiation therapy or laminectomy to treat the cord compression [169]. Learning disabilities are also a potential secondary complication from neuroblastoma treatment, in addition to psychosocial late effects such as low income and lower rates of employment [165].…”