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Takita J. Molecular Basis and Clinical Features of Neuroblastoma. JMA J. 2021;4(4):321-331doi: 10.31662/jmaj.2021-0077.
Takita, J. (2021). Molecular Basis and Clinical Features of Neuroblastoma. JMA journal, 4(4), 321-331. https://doi.org/10.31662/jmaj.2021-0077
Takita, Junko. "Molecular Basis and Clinical Features of Neuroblastoma." JMA journal vol. 4,4 (2021): 321-331. doi: https://doi.org/10.31662/jmaj.2021-0077
Takita J. Molecular Basis and Clinical Features of Neuroblastoma. JMA J. 2021 Oct 15;4(4):321-331. doi: 10.31662/jmaj.2021-0077. Epub 2021 Sep 01. PMID: 34796286; PMCID: PMC8580727.
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JMA J. 2021 Oct 15;4(4):321-331. doi: 10.31662/jmaj.2021-0077. Epub 2021 Sep 01.

Molecular Basis and Clinical Features of Neuroblastoma.

JMA journal

Junko Takita

Affiliations

  1. Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

PMID: 34796286 PMCID: PMC8580727 DOI: 10.31662/jmaj.2021-0077

Abstract

Neuroblastoma, a neoplasm of the sympathetic nervous system, originates from neuroblastoma stem cells during embryogenesis. It exhibits unique clinical features including a tendency for spontaneous regression of tumors in infants and a high frequency of metastatic disease at diagnosis in patients aged over 18 months. Genetic risk factors and epigenetic dysregulation also play a significant role in the development of neuroblastoma. Over the past decade, our understanding of this disease has advanced considerably. This has included the identification of chromosomal copy number aberrations specific to neuroblastoma development, risk groups, and disease stage. However, high-risk neuroblastoma remains a therapeutic challenge for pediatric oncologists. New therapeutic approaches have been developed, either as alternatives to conventional chemotherapy or in combination, to overcome the dismal prognosis. Particularly promising strategies are targeted therapies that directly affect cancer cells or cancer stem cells while exhibiting minimal effect on healthy cells. This review summarizes our understanding of neuroblastoma biology and prognostic features and focuses on novel therapeutic strategies for this intractable disease.

Copyright © Japan Medical Association.

Keywords: MYCN; anaplastic lymphoma kinase (ALK); chromosomal copy number alterations; neuroblastoma; spontaneous regression

Conflict of interest statement

None

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