NUT carcinoma in children, adolescents and young adults.
Bromodomain and Extra-Terminal (BET) protein
Children
Histone deacetylase
Midline carcinoma
NUT carcinoma
Rare tumour
Targeted therapies
Journal
Bulletin du cancer
ISSN: 1769-6917
Titre abrégé: Bull Cancer
Pays: France
ID NLM: 0072416
Informations de publication
Date de publication:
Apr 2022
Apr 2022
Historique:
received:
08
06
2021
revised:
22
01
2022
accepted:
31
01
2022
pubmed:
17
4
2022
medline:
7
5
2022
entrez:
16
4
2022
Statut:
ppublish
Résumé
NUT carcinoma (NC), defined by the presence of the NUTM1 rearrangement, is an aggressive tumour associated with poor prognosis. This rare cancer is underdiagnosed and difficult to treat. The primary objective of this review is to describe the clinical, radiological and laboratory features of NC in young patients. The secondary objective is to propose a consensual strategy for the French very Rare Tumour group (FRACTURE group). NUT-specific antibody immunostaining in cases of undifferentiated or poorly differentiated carcinoma may demonstrate the specific NUT gene rearrangement. NCs are frequently advanced stage at diagnosis and the outcome remains poor despite a global strategy that generally includes conventional combination chemotherapy with wide local therapy (surgery, radiotherapy). Chemosensitivity is frequently only transient. Recent data have shown that new targeted drugs (histone deacetylase and bromodomain and extra-terminal protein inhibitors) are promising, but their role has yet to be evaluated in NC. Centralized data review is necessary to improve our knowledge of paediatric NC. We propose a multimodal strategy based on published data and their personal experience.
Sections du résumé
BACKGROUND
BACKGROUND
NUT carcinoma (NC), defined by the presence of the NUTM1 rearrangement, is an aggressive tumour associated with poor prognosis. This rare cancer is underdiagnosed and difficult to treat.
OBJECTIVE AND METHODS
OBJECTIVE
The primary objective of this review is to describe the clinical, radiological and laboratory features of NC in young patients. The secondary objective is to propose a consensual strategy for the French very Rare Tumour group (FRACTURE group).
RESULTS
RESULTS
NUT-specific antibody immunostaining in cases of undifferentiated or poorly differentiated carcinoma may demonstrate the specific NUT gene rearrangement. NCs are frequently advanced stage at diagnosis and the outcome remains poor despite a global strategy that generally includes conventional combination chemotherapy with wide local therapy (surgery, radiotherapy). Chemosensitivity is frequently only transient.
CONCLUSION
CONCLUSIONS
Recent data have shown that new targeted drugs (histone deacetylase and bromodomain and extra-terminal protein inhibitors) are promising, but their role has yet to be evaluated in NC. Centralized data review is necessary to improve our knowledge of paediatric NC. We propose a multimodal strategy based on published data and their personal experience.
Identifiants
pubmed: 35428456
pii: S0007-4551(22)00074-1
doi: 10.1016/j.bulcan.2022.01.015
pii:
doi:
Substances chimiques
Nuclear Proteins
0
Transcription Factors
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
491-504Informations de copyright
Copyright © 2022 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.