Neuroblastoma: From Diagnosis to Treatment

Neuroblastoma (NB) is the most common extracranial solid tumor in children. It affects mostly pre-teen age, and in almost 50% of the cases the disease is already metastatic at initial diagnosis. NB originates from neuroectodermal cells of the neural crest that migrate during the embryonic life, giving rise to the sympathetic ganglia and adrenal medulla. For this reason, over 90% of patients with NB show high levels of catecholamines in the serum and urine. Typically, symptoms at presentation reflect the location and the extent of the disease, as well as the secretory status of the tumor. The diagnosis of NB is based on a multidisciplinary workup including clinical and laboratory data, tumor histology, bone marrow biopsy, and instrumental investigations. A significant role in the diagnostic and therapeutic process of NB is nowadays played by metaiodobenzylguanidine (MIBG) scintigraphy, considered as standard for the initial staging, detection of recurrence and disease monitoring. According to age, disease stage, tumor histology, N - MYC gene amplification, or chromosomal aberrations, NB can be classified into four different risk groups, reflecting different prognosis and leading to different therapeutic options. The treatment of NB is based on a variegate approach ranging from pure monitoring in very low-risk patients, to curative surgery or combined surgery and chemotherapy in low-intermediate risk, to very aggressive multi-agent regimens for high risk patients.