Neuroblastoma (Peripheral neuroblastic tumours). (November 2016)

• Record Type: Journal Article
• Authors: Luksch, Roberto; Castellani, Maria Rita; Collini, Paola; De Bernardi, Bruno; Conte, Massimo; Gambini, Claudio; Gandola, Lorenza; Garaventa, Alberto; Biasoni, Davide; Podda, Marta; Sementa, Angela Rita; Gatta, Gemma; Tonini, Gian Paolo
• Is Part Of: Critical reviews in oncology/hematology. Volume 107(2016)
• Page Start: 163
• Page End: 181
• Publication Date: 2016-11
• Subjects: Childhood tumours -- Peripheral sympathetic system -- Peripheral neuroblastic tumours -- Neuroblastoma; Oncology -- Periodicals; Hematology -- Periodicals; 616.994
• DOI: 10.1016/j.critrevonc.2016.10.001
• Languages: English
• ISSNs: 1040-8428

Abstract

Abstract: Peripheral neuroblastic tumours (PNTs), a family of tumours arising in the embryonal remnants of the sympathetic nervous system, account for 7–10% of all tumours in children. In two-thirds of cases, PNTs originate in the adrenal glands or the retroperitoneal ganglia. At least one third present metastases at onset, with bone and bone marrow being the most frequent metastatic sites. Disease extension, MYCN oncogene status and age are the most relevant prognostic factors, and their influence on outcome have been considered in the design of the recent treatment protocols. Consequently, the probability of cure has increased significantly in the last two decades. In children with localised operable disease, surgical resection alone is usually a sufficient treatment, with 3-year event-free survival (EFS) being greater than 85%. For locally advanced disease, primary chemotherapy followed by surgery and/or radiotherapy yields an EFS of around 75%. The greatest problem is posed by children with metastatic disease or amplified MYCN gene, who continue to do badly despite intensive treatments. Ongoing trials are exploring the efficacy of new drugs and novel immunological approaches in order to save a greater number of these patients.