Neuroblastoma, the most common extracranial solid tumor in children, presents a clinical paradox: some tumors regress spontaneously, while others prove relentlessly aggressive. A new narrative review, published January 6, 2026, in the World Journal of Pediatric Surgery, synthesizes current evidence to offer a risk-guided framework for managing this unpredictable disease. The review, authored by specialists from the Royal Hospital for Children in Glasgow and the University of Liverpool, underscores that treatment decisions must integrate tumor stage, patient age, histology, chromosomal aberrations, and molecular markers such as MYCN amplification.
The stakes are high. Neuroblastoma accounts for about 15% of pediatric cancer deaths, with five-year survival exceeding 90% for low- and intermediate-risk cases but falling below 60% for high-risk disease. Clinicians must therefore balance treatment intensity against surgical risk, organ preservation, and long-term toxicity. The review, with DOI:10.1136/wjps-2025-001127, provides a practical roadmap for multidisciplinary teams.
Risk assessment begins with diagnosis. About 70% of patients present with abdominal disease, and workup typically includes urine catecholamines, MRI, MIBG scintigraphy, bone marrow evaluation, biopsy, and genetic profiling. The International Neuroblastoma Risk Group Staging System (INRGSS) uses imaging-defined risk factors to classify disease before treatment. Molecular profiling adds critical information: MYCN amplification, present in roughly a quarter of tumors and 40%–50% of high-risk cases, signals aggressive behavior.
Treatment strategies range from observation alone in selected infants to intensive multimodal therapy for high-risk disease. The review cites a prospective study showing 10-year event-free survival of 94.7% and overall survival of 97.4% in carefully selected infants managed with observation, supporting this approach when strict criteria are met. For high-risk disease, chemotherapy, surgery, myeloablative therapy, stem cell rescue, radiotherapy, GD2-targeting antibodies, and retinoic acid are employed. The authors emphasize that surgery is one component of the pathway, not an isolated technical goal.
The review also addresses unresolved controversies. Computed tomography (CT) may better delineate surgical anatomy in some high-risk abdominal tumors than MRI, but the survival benefit of more extensive resection remains debated. Standardized surgical reporting could improve trial comparisons, especially where the extent of resection is uncertain. Emerging therapies, including GD2 antibodies, CAR T-cell therapy, ALK inhibitors, and telomere biology modifiers, point toward more personalized treatment.
Beyond survival, the authors stress the importance of long-term follow-up for survivors, including monitoring for fertility issues, hearing loss, endocrine dysfunction, cognitive deficits, and secondary cancers. This holistic perspective is crucial as more children survive neuroblastoma. The review's risk-based framework supports consistent decisions about when to observe, biopsy, operate, or intensify therapy, and it highlights the need for structured surgical reports and international collaboration.
The review, published in the World Journal of Pediatric Surgery, an open-access journal indexed in PubMed and Scopus, offers an updated reference for surgeons, oncologists, radiologists, and pathologists. By integrating diagnosis, risk classification, and treatment into a single framework, it aims to improve decision-making and outcomes for children with neuroblastoma.
