Extremely high prognostic risk group of neuroblastic tumors: histological, immunophenotypic and genetic characteristics. Literature review and own observations

Abstract

Clinical heterogeneity appears to be one of the most characteristic feature of the group of peripheral neuroblastic tumors, ranging from spontaneous tumor regression to a widespread process, often resistant to multimodal therapeutic strategies. Despite significant progress in treatment, about 40 % of patients with high-risk neuroblastoma die from disease recurrence after complete response to first-line therapy. These 40 % are considered a “extremely high” risk group requiring intensification of therapeutic regimens from the time of diagnosis. Histological and molecular predictive features of this group are of high scientific and practical interest for the correct therapy.