Tags

Type your tag names separated by a space and hit enter

Emerging entities in NUTM1-rearranged neoplasms.
Genes Chromosomes Cancer. 2020 Jun; 59(6):375-385.GC

Abstract

Structural alterations of NUTM1 were originally thought to be restricted to poorly differentiated carcinomas with variable squamous differentiation originating in the midline organs of children and adolescents. Termed NUT carcinomas (NCs), they were defined by a t(15;19) chromosomal rearrangement that was found to result in a BRD4-NUTM1 gene fusion. However, the use of DNA and RNA-based next-generation sequencing has recently revealed a multitude of new NUTM1 fusion partners in a diverse array of neoplasms including sarcoma-like tumors, poromas, and acute lymphoblastic leukemias (ALLs) that we propose to call NUTM1-rearranged neoplasms (NRNs). Intriguingly, the nosology of NRNs often correlates with the functional classification of the fusion partner, suggesting different oncogenic mechanisms within each NRN division. Indeed, whereas NCs are characterized by their aggressiveness and intransigence to standard therapeutic measures, the more positive clinical outcomes seen in some sarcoma and ALL NRNs may reflect these mechanistic differences. Here we provide a broad overview of the molecular, nosological, and clinical features in these newly discovered neoplastic entities. We describe how aberrant expression of NUTM1 due to fusion with an N-terminal DNA/chromatin-binding protein can generate a potentially powerful chromatin modifier that can give rise to oncogenic transformation in numerous cellular contexts. We also conclude that classification, clinical behavior, and therapeutic options may be best defined by the NUTM1 fusion partner rather than by tumor morphology or immunohistochemical profile.

Authors+Show Affiliations

Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.Department of Pathology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

32060986

Citation

McEvoy, Christopher R., et al. "Emerging Entities in NUTM1-rearranged Neoplasms." Genes, Chromosomes & Cancer, vol. 59, no. 6, 2020, pp. 375-385.
McEvoy CR, Fox SB, Prall OWJ. Emerging entities in NUTM1-rearranged neoplasms. Genes Chromosomes Cancer. 2020;59(6):375-385.
McEvoy, C. R., Fox, S. B., & Prall, O. W. J. (2020). Emerging entities in NUTM1-rearranged neoplasms. Genes, Chromosomes & Cancer, 59(6), 375-385. https://doi.org/10.1002/gcc.22838
McEvoy CR, Fox SB, Prall OWJ. Emerging Entities in NUTM1-rearranged Neoplasms. Genes Chromosomes Cancer. 2020;59(6):375-385. PubMed PMID: 32060986.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Emerging entities in NUTM1-rearranged neoplasms. AU - McEvoy,Christopher R, AU - Fox,Stephen B, AU - Prall,Owen W J, Y1 - 2020/02/25/ PY - 2019/12/08/received PY - 2020/02/03/revised PY - 2020/02/07/accepted PY - 2020/2/16/pubmed PY - 2020/2/16/medline PY - 2020/2/16/entrez KW - ALL KW - NUT carcinoma KW - NUTM1 KW - gene fusion KW - sarcoma SP - 375 EP - 385 JF - Genes, chromosomes & cancer JO - Genes Chromosomes Cancer VL - 59 IS - 6 N2 - Structural alterations of NUTM1 were originally thought to be restricted to poorly differentiated carcinomas with variable squamous differentiation originating in the midline organs of children and adolescents. Termed NUT carcinomas (NCs), they were defined by a t(15;19) chromosomal rearrangement that was found to result in a BRD4-NUTM1 gene fusion. However, the use of DNA and RNA-based next-generation sequencing has recently revealed a multitude of new NUTM1 fusion partners in a diverse array of neoplasms including sarcoma-like tumors, poromas, and acute lymphoblastic leukemias (ALLs) that we propose to call NUTM1-rearranged neoplasms (NRNs). Intriguingly, the nosology of NRNs often correlates with the functional classification of the fusion partner, suggesting different oncogenic mechanisms within each NRN division. Indeed, whereas NCs are characterized by their aggressiveness and intransigence to standard therapeutic measures, the more positive clinical outcomes seen in some sarcoma and ALL NRNs may reflect these mechanistic differences. Here we provide a broad overview of the molecular, nosological, and clinical features in these newly discovered neoplastic entities. We describe how aberrant expression of NUTM1 due to fusion with an N-terminal DNA/chromatin-binding protein can generate a potentially powerful chromatin modifier that can give rise to oncogenic transformation in numerous cellular contexts. We also conclude that classification, clinical behavior, and therapeutic options may be best defined by the NUTM1 fusion partner rather than by tumor morphology or immunohistochemical profile. SN - 1098-2264 UR - https://www.unboundmedicine.com/medline/citation/32060986/Emerging_entities_in_NUTM1-rearranged_neoplasms L2 - https://doi.org/10.1002/gcc.22838 DB - PRIME DP - Unbound Medicine ER -
Try the Free App:
Prime PubMed app for iOS iPhone iPad
Prime PubMed app for Android
Prime PubMed is provided
free to individuals by:
Unbound Medicine.