Neuroblastoma (Peripheral neuroblastic tumours)

Roberto Luksch, Maria Rita Castellani, Paola Collini, Bruno De Bernardi, Massimo Conte, Claudio Gambini, Lorenza Gandola, Alberto Garaventa, Davide Biasoni, Marta Podda, Angela Rita Sementa, Gemma Gatta, Gian Paolo Tonini

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)163-181
Number of pages19
JournalCritical Reviews in Oncology/Hematology
Volume107
DOIs
Publication statusPublished - Nov 2016

Keywords

  • Review
  • Journal Article

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