Neuroblastoma (Peripheral neuroblastic tumours).
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.
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: roberto.luksch@istitutotumori.mi.it.
Roberto
R
Luksch
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Maria Rita
MR
Castellani
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Paola
P
Collini
Giannina Gaslini Children's Research Hospital, Genoa, Italy.
Bruno
B
De Bernardi
Giannina Gaslini Children's Research Hospital, Genoa, Italy.
Massimo
M
Conte
Giannina Gaslini Children's Research Hospital, Genoa, Italy.
Claudio
C
Gambini
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Lorenza
L
Gandola
Giannina Gaslini Children's Research Hospital, Genoa, Italy.
Alberto
A
Garaventa
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Davide
D
Biasoni
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Marta
M
Podda
Giannina Gaslini Children's Research Hospital, Genoa, Italy.
Angela Rita
AR
Sementa
Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Gemma
G
Gatta
Neuroblastoma Laboratory, Paediatric Research Institute, Padua, Italy.
Gian Paolo
GP
Tonini
D000818:Animals; D015972:Gene Expression Regulation, Neoplastic; D015894:Genome, Human; D005838:Genotype; D006801:Humans; D009447:Neuroblastoma / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000473:pathology; D010641:Phenotype; D015995:Prevalence
Childhood tumours; Neuroblastoma; Peripheral neuroblastic tumours; Peripheral sympathetic system