MYCN Function in Neuroblastoma Development

Jörg Otte; Cecilia Dyberg; Adena Pepich; John Inge Johnsen

doi:10.3389/fonc.2020.624079

MYCN Function in Neuroblastoma Development

Front Oncol. 2021 Jan 27:10:624079. doi: 10.3389/fonc.2020.624079. eCollection 2020.

Authors

Jörg Otte¹, Cecilia Dyberg¹, Adena Pepich¹, John Inge Johnsen¹

Affiliation

¹ Childhood Cancer Research Unit, Department of Children's and Women's Health, Karolinska Institutet, Stockholm, Sweden.

Abstract

Dysregulated expression of the transcription factor MYCN is frequently detected in nervous system tumors such as childhood neuroblastoma. Here, gene amplification of MYCN is a single oncogenic driver inducing neoplastic transformation in neural crest-derived cells. This abnormal MYCN expression is one of the strongest predictors of poor prognosis. It is present at diagnosis and is never acquired during later tumorigenesis of MYCN non-amplified neuroblastoma. This suggests that increased MYCN expression is an early event in these cancers leading to a peculiar dysregulation of cells that results in embryonal or cancer stem-like qualities, such as increased self-renewal, apoptotic resistance, and metabolic flexibility.

Keywords: MYCN; cancer stem cell; childhood cancer; neural crest; neuroblastoma.

Publication types

Review