Neuroblastoma (NB) is a paediatric tumour derived from primordial neural crest cells that normally differentiate into the sympathetic nervous system. About 50 % of patients have metastatic disease at presentation, with a propensity to involve the bone marrow and cortical bone. Functional imaging with 123I-MIBG scintigraphy is an essential tool in patients with NB both for initial staging and response to therapy, allowing visualization of the primary tumour and metastatic lesions in the various sites. Various radiopharmaceuticals for positron emission tomography (PET) such as fluorine-18-fluorodeoxyglucose (18F-FDG), fluorine-18-dihydroxyphenylalanine (18F-DOPA), 68Ga-labelled somatostatin analogues, 11C-hydroxyephedrine (11C-HED) and 124I-MIBG are currently under investigation. Treatment protocols for NB are stratified according to risk, which is defined on the basis of biologic and clinical prognostic factors. Treatment of high-risk NB continues to be a challenge, with a high rate of relapse in the bone and bone marrow. Since its initial application, the role of 131I-MIBG in the treatment of high-risk NB evolved with time. Palliation of symptoms is achieved in most of the patients with advanced/resistant NB. Used as monotherapy 131I-MIBG gives about 30 % of objective responses. Myelotoxicity is the only significant toxic effect. To achieve greater antitumour efficacy and low toxicity to normal tissues, various treatment regimens including 131I-MIBG in combination with chemotherapy or other agents have been designed over time with encouraging results.
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