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Neuroblastoma: a master of disguise and a challenge to cure

Neuroblastoma: a master of disguise and a challenge to cure
Neuroblastoma: a master of disguise and a challenge to cure
Neuroblastoma is one of the commonest childhood cancers and typically affects very young children. It is characterized by a very broad spectrum of clinical presentation and outcome, driven by the biology of the tumour. This ranges from ‘low risk’ tumours, most commonly found in infants, which may spontaneously regress and have an excellent prognosis with minimal or no treatment, to ‘high risk’ disease, which carries a poor prognosis despite intensive multi-modal treatment. Although particular presentations may be associated with favourable or unfavourable outcome, the clinical features may mask the underlying biology of the tumour and a full assessment of the clinical and biological features is required to determine appropriate treatment. The International Neuroblastoma Risk Group classification, based on the age of the patient and the stage, grade and genetics of the tumour, is used to stratify treatment according to risk factors. This review provides an overview of current neuroblastoma management, focusing on how classification is applied in practice, and how this is used to determine individual patient treatment. The challenges that remain in treating patients with high-risk disease are discussed.
IDRF, INRG, International Neuroblastoma Risk Group, MYC-N, SCA, image defined risk factor, metastatic, risk stratification, segmental chromosomal abnormalities
1751-7222
171 - 177
Calderoni, Debora
e8f8139a-bd38-46ed-8717-28513a618447
Barone, Giuseppe
f050af49-028a-4283-803e-6e1ac2929d4a
Elliot, Martin
e230b88c-2c52-4f65-8c00-0893099d3512
Tweedle, Deborah A.
185dd5ae-fea0-4ed2-8305-794b8b0055d2
Ramanujachar, Ramya
9a27f52b-157d-4349-b255-a2990da1e986
Gray, Juliet
12d5e17c-97bb-4d6d-8fc4-3914b730ed42
Calderoni, Debora
e8f8139a-bd38-46ed-8717-28513a618447
Barone, Giuseppe
f050af49-028a-4283-803e-6e1ac2929d4a
Elliot, Martin
e230b88c-2c52-4f65-8c00-0893099d3512
Tweedle, Deborah A.
185dd5ae-fea0-4ed2-8305-794b8b0055d2
Ramanujachar, Ramya
9a27f52b-157d-4349-b255-a2990da1e986
Gray, Juliet
12d5e17c-97bb-4d6d-8fc4-3914b730ed42

Calderoni, Debora, Barone, Giuseppe, Elliot, Martin, Tweedle, Deborah A., Ramanujachar, Ramya and Gray, Juliet (2022) Neuroblastoma: a master of disguise and a challenge to cure. Paediatrics and Child Health, 32 (5), 171 - 177. (doi:10.1016/j.paed.2022.02.001).

Record type: Review

Abstract

Neuroblastoma is one of the commonest childhood cancers and typically affects very young children. It is characterized by a very broad spectrum of clinical presentation and outcome, driven by the biology of the tumour. This ranges from ‘low risk’ tumours, most commonly found in infants, which may spontaneously regress and have an excellent prognosis with minimal or no treatment, to ‘high risk’ disease, which carries a poor prognosis despite intensive multi-modal treatment. Although particular presentations may be associated with favourable or unfavourable outcome, the clinical features may mask the underlying biology of the tumour and a full assessment of the clinical and biological features is required to determine appropriate treatment. The International Neuroblastoma Risk Group classification, based on the age of the patient and the stage, grade and genetics of the tumour, is used to stratify treatment according to risk factors. This review provides an overview of current neuroblastoma management, focusing on how classification is applied in practice, and how this is used to determine individual patient treatment. The challenges that remain in treating patients with high-risk disease are discussed.

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Neuroblastoma - review 31-12-21 - Accepted Manuscript
Restricted to Repository staff only until 6 April 2023.
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Neuroblastoma_ a master of disguise and a challenge to cure _ Elsevier Enhanced Reader - Version of Record
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More information

e-pub ahead of print date: 6 April 2022
Published date: 2 May 2022
Additional Information: Publisher Copyright: © 2022 Elsevier Ltd
Keywords: IDRF, INRG, International Neuroblastoma Risk Group, MYC-N, SCA, image defined risk factor, metastatic, risk stratification, segmental chromosomal abnormalities

Identifiers

Local EPrints ID: 457236
URI: http://eprints.soton.ac.uk/id/eprint/457236
ISSN: 1751-7222
PURE UUID: 3d4e1dcc-e64d-4ffa-853b-90e4da5c6759
ORCID for Juliet Gray: ORCID iD orcid.org/0000-0002-5652-4722

Catalogue record

Date deposited: 26 May 2022 16:58
Last modified: 24 Jul 2022 01:38

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Contributors

Author: Debora Calderoni
Author: Giuseppe Barone
Author: Martin Elliot
Author: Deborah A. Tweedle
Author: Ramya Ramanujachar
Author: Juliet Gray ORCID iD

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