Abstract

Neuroblastoma is the most common solid tumor of the childhood. The 5-year-survival rates increased over 50% during the last few decades. Genomic studies demonstrated recurrent mutations of ALK (9.2%), PTPN11 (2.9%), ATRX (2.5%), Nmyc (1.7%) and NRAS (0.8%) genes in children with neuroblastoma. The staging systems based on the evaluation of the extent of surgical excision (International Neuroblastoma Staging System - INSS), and risk groups (International Neuroblastoma Risk Group Staging System-INRGSS) have been replaced by a new risk group pretreatment classification system (International Risk Group Pretreatment Classification System) which takes radiological risk factors, age of the patient, histological type and maturation status “Nmyc and 11q site”, and ploidy of the tumor at the time of diagnosis into consideration but excludes extent of surgery, Surgical resection is the main mode of treatment in patients with localized tumor, and low- or intermediate-risk disease. Surgical resection is usually performed at the end of induction therapy in patients with advanced or high-risk disease and helps local control of the disease. Although evidence retrieved from retrospective studies, and overall evaluation of their meta-analysis do not provide strong evidence, they demonstrate that complete or gross total resection of the tumor at least prolongs eventfree survival rates in children with advanced stages of neuroblastoma. In the light of updated information, surgeons should take image-defined risk factors and their personal experiences into consideration when deciding resectability of the tumor, and favour multidisciplinary approach.