Childhood neuroblastoma: case presentation and systematic review of the literature

Abstract BACKGROUND. Olfactory neuroblastoma (ONB) is a rare tumor in children, with locally aggressive behaviour and difficult surgical access. OBJECTIVE. To analyze the clinical, therapeutic, and prognostic characteristics of pediatric ONB through a systematic review of published clinical cases and the presentation of a maxillary case. MATERIAL AND METHODS. A systematic review was carried out in accordance with the PRISMA guidelines. The search was conducted in PubMed, Scopus, ScienceDirect and Mendeley, including studies published in English up to December 2024. The following descriptors were used: (“esthesioneuroblastoma” AND “neuroblastoma”) AND (“children” OR “child” OR “pediatric”), applying filters by age (<18 years) and clinical case design or case series. Two authors made the selection blindly using Rayyan. Animal studies with no individual description or duplicates were excluded. Methodological quality was evaluated using the CARE guideline. Additionally, a case of maxillary ONB was documented in a 3-year-old girl. RESULTS. 76 patients were analyzed. The most effective therapeutic strategies were multimodal, combining chemotherapy, radiotherapy and surgery. The disease-free survival rate was 61.8%. The clinical case showed a favourable evolution after surgical debulking and neoadjuvant chemotherapy. CONCLUSION. ONB in the pediatric population requires multidisciplinary management. Strengthening collaborative registries and standardising diagnostic and therapeutic protocols is proposed to improve clinical evidence and long-term results.