Treatment of Spinal Involvement in Neuroblastoma Patients

Abstract

Introduction: Considerable controversy exists regarding the appropriate management of spinal involvement in neuroblastoma (NB) patients. We review a large group of such patients and offer treatment recommendations. Methods: Forty-six patients with epidural and/or neural foraminal involvement treated between 1987 and 1998 were staged according to the International NB Staging System (INSS) and classified as high-risk (INSS stage 4; n = 31) or low-risk (INSS stage <4; n = 15). Of 13 high- risk patients with normal neurologic examinations and no radiographic high-grade spinal cord compression (HGSCC), 12 were treated initially with chemotherapy, and only 1 demonstrated neurologic deterioration. HGSCC was present in 18 patients with high-risk NB; 7 of 10 (70%) treated initially with chemotherapy and 6 of 6 (100%) managed initially with operation improved or remained stable. All 9 low-risk patients with normal neurologic examinations and no HGSCC remained neurologically intact following operations (n = 7) or chemotherapy (n = 2). All 4 low-risk patients with HGSCC treated with operations improved or remained stable, and 0 of 2 (0%) low-risk patients treated initially with chemotherapy remained stable. Spinal deformities occurred in 2 of 16 patients (12.5%) treated nonoperatively and in 9 of 30 (30.0%) who underwent operations. Conclusions: High-risk NB patients with spinal involvement but normal neurologic examinations should be offered chemotherapy. High-risk patients with HGSCC may respond to chemotherapy, but a small percentage will require operations for progressive neurologic deficits. Chemotherapy may be avoided in low-risk patients who are offered potentially curative operations. Patients treated with operations for epidural disease are at high risk of subsequently developing spinal deformity.

Primary osseous tumors of the pediatric spinal column: review of pathology and surgical decision making

“…Parikh et al 79 reported on 22 patients, 19 of whom attained a favorable outcome and long-term survival. Sandberg et al 87 concluded that high-risk neuroblastoma patients with high-grade spinal cord compression may respond to chemotherapy, but a percentage will require surgical decompression for progressive neurological deficits. For patients at low risk, the 5-year survival rate is greater than 95%; for those in the intermediate-risk group, the rate is between 90% and 95%; and for those in the high-risk group, the rate is between 40% and 50%.…”

Neuroblastoma with spinal cord compression: is there an emergency treatment of choice?

“…In the last decade, researchers have used a variety of techniques to study brain reorganization in children with cerebral palsy, including functional magnetic resonance imaging, magnetoencephalography (MEG), transcranial magnetic simulation, diffusion tensor imaging, and near infrared spectroscopy. 1 These studies (like the MEG study by Nevalainen et al 5 ) help us understand cortical reorganization in cerebral palsy and provides a glimpse of the clinical consequences of neuroplasticity processes.…”

“…There is considerable debate about advantages and disadvantages of these treatment options and treatment algorithms vary among institutions. [2][3][4][5] In the largest cohort of children with neuroblastoma and spinal cord compression published so far (n=122), Simon et al 6 try to clarify the controversial issues about the emergency treatment of choice in these children. To achieve this aim, the authors retrospectively analyzed the short-and longterm outcomes of patients with neuroblastoma and spinal cord compression included in four consecutive prospective German clinical trials.…”

“…2 Additional studies, such as the Nevalainen et al MEG study, 5 have examined cortical somatosensory reorganization in children with cerebral palsy. Such studies suggest that somatosensory representation of the affected limb can be represented bilaterally, by both the affected and unaffected hemispheres, 5 although other patterns of somatosensory reorganization have been reported.…”

“…2 Additional studies, such as the Nevalainen et al MEG study, 5 have examined cortical somatosensory reorganization in children with cerebral palsy. Such studies suggest that somatosensory representation of the affected limb can be represented bilaterally, by both the affected and unaffected hemispheres, 5 although other patterns of somatosensory reorganization have been reported. 4,5 Despite the information gained from these studies, understanding the interaction between reorganization of the motor and somatosensory systems, and which patterns of reorganization facilitate or inhibit development of motor function is still limited.…”