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.
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