Neuroblastoma is the most common extracranial solid tumor of childhood. It originates from precursor cells of the peripheral nervous system in the adrenal medulla or the sympathetic chain. Approximately 120 new cases are diagnosed annually in Germany, representing 5-6% of all childhood cancers. The disease is characterized by marked biological heterogeneity, with outcomes ranging from spontaneous regression and localized tumors to metastatic, treatment-refractory high-risk disease. Common metastatic sites include the bone marrow, bone, lymph nodes, liver, lungs, and the central nervous system. Diagnosis and risk stratification are based on clinical and molecular features, including MYCN and ALK status, chromosome 1p36 aberrations, and tumor RNA expression profiles. Treatment is risk adapted: low-risk patients are managed with observation or surgery alone and receive chemotherapy only in the case of progressive disease or threatening symptoms. Intermediate-risk patients receive chemotherapy, surgery, and in some cases radiotherapy. High-risk patients undergo intensive multimodal treatment consisting of induction chemotherapy, surgery, high-dose chemotherapy with autologous stem cell transplantation, radiotherapy, and anti-GD2 immunotherapy. Despite successful first-line treatment, similar to 50% of patients relapse, and similar to 80% of these patients experience further relapse or disease progression. The 5-year overall survival rate for high-risk neuroblastoma remains only similar to 50%. Current clinical trials are evaluating chemoimmunotherapy in frontline and relapsed settings. Other promising approaches include ALK inhibitors for ALK-positive tumors, stem cell transplantation combined with anti-GD2 immunotherapy, chimeric antigen receptor (CAR) T cell therapy, and peptide vaccination. Diagnostic research focuses on liquid biopsy and F-18-meta-fluorobenzylguanidine positron-emission tomography computed tomography ([F-18]mFBG PET/CT) imaging.
