The Changing Role of Radiation Therapy in the Treatment of Neuroblastoma.

Abstract

Neuroblastoma (NBL) is the fourth most common pediatric malignancy. With a median age at diagnosis of 2 years, it represents half of all malignancies that present in the first month of life and one third of those that are diagnosed in the first year of life. NBL is unique among human cancers in its ability to undergo spontaneous differentiation and permanent tumor regression. This phenomenon is particularly characteristic of disseminated disease with liver, skin, and limited bone marrow involvement and involving a limited primary and presenting in children under 1 year of age. Advances in the understanding of the biologic behavior of NBL coupled with the clinical presentation have led to a risk-based approach to treatment, minimizing the treatment to some patients and supporting the need for more aggressive treatment to others. A new international staging system has been adopted that has allowed better comparisons among treatment reports from varying centers and cooperative groups. This article reviews the risk-related approach to the treatment of NBL and the changing role of radiation therapy.