Low and Intermediate Risk Disease

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The standard of care for irradiation in neuroblas-toma began to evolve in the 1980s, when studies indicated that patients with early-stage disease did not benefit from post-operative radiation to the primary site. In the 1970s and 1980s two large randomized trials focused on localized and regional disease, and although neither study examined radiation in a prospective manner, mounting evidence suggested that adjuvant radiation therapy did not influence survival of patients with stage-2 neuroblastoma (Evans et al. 1976,1984; Matthay et al. 1989).

The CCG recently reported on the outcome of Evans stage 1-2 neuroblastoma treated with surgery as primary therapy. Chemotherapy and radiation were reserved for progressive or recurrent disease or local disease-related symptoms such as spinal cord compression or respiratory distress. The event-free survival (EFS) and overall survival (OS) rates were 93 and 99%, respectively, for stage-1 patients, and 81 and 98%, respectively, for stage-2 patients. Additional therapy (radiation, surgery, and/or chemotherapy) was needed in only 10% of stage-1 patients and 20% of stage-2 patients (Perez et al. 2000).

Based on these results, the current standard of care is to reserve radiation therapy only for those low-risk patients whose disease is not adequately controlled with surgery and chemotherapy. In the current COG protocol for low-risk neuroblastoma (#P9641), a dose of 21 Gy is recommended for stage-1 and stage-2 patients who require radiotherapy.

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