NMC is a recently recognized cancer type hallmarked by chromosomal translocation involving the NUT gene, a catastrophic event leading to fusion oncoprotein responsible for malignant transformation and tumor progression. The aggressiveness of disease together with a poor response to conventional treatment make NMC one of the most lethal cancer. Moreover, although until recently NMC has been poorly understood and largely neglected, the number of reported cases is steadily rising. Recently, in addition to its pathogenetic and diagnostic role, NUT-fusion oncoprotein has been shown to be amenable to targeted inhibition using BET inhibitors. Future clinical trials are warranted with the aim of investigate the incorporation of targeted agent into multimodal therapeutic strategy. Since new promising NUT-targeting drugs are emerging that may affect the clinical course, the correct and prompt recognition of NMC is key to improve patients' outcome.
NUT midline carcinoma: Current concepts and future perspectives of a novel tumour entity / Salati, M.; Baldessari, C.; Bonetti, L. R.; Messina, C.; Merz, V.; Cerbelli, B.; Botticelli, A.. - In: CRITICAL REVIEWS IN ONCOLOGY HEMATOLOGY. - ISSN 1040-8428. - 144:(2019), pp. 1-6. [10.1016/j.critrevonc.2019.102826]
NUT midline carcinoma: Current concepts and future perspectives of a novel tumour entity
Bonetti L. R.;
2019
Abstract
NMC is a recently recognized cancer type hallmarked by chromosomal translocation involving the NUT gene, a catastrophic event leading to fusion oncoprotein responsible for malignant transformation and tumor progression. The aggressiveness of disease together with a poor response to conventional treatment make NMC one of the most lethal cancer. Moreover, although until recently NMC has been poorly understood and largely neglected, the number of reported cases is steadily rising. Recently, in addition to its pathogenetic and diagnostic role, NUT-fusion oncoprotein has been shown to be amenable to targeted inhibition using BET inhibitors. Future clinical trials are warranted with the aim of investigate the incorporation of targeted agent into multimodal therapeutic strategy. Since new promising NUT-targeting drugs are emerging that may affect the clinical course, the correct and prompt recognition of NMC is key to improve patients' outcome.
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