French, Christopher A., Cheng, Michael L., Hanna, Glenn J., DuBois, Steven G., Chau, Nicole G., Hann, Christine L., Storck, Simone, Salgia, Ravi, Trucco, Matteo, Tseng, Jennifer, Stathis, Anastasios, Piekarz, Richard, Lauer, Ulrich M., Massard, Christophe, Bennett, Kelly, Coker, Shodeinde, Tontsch-Grunt, Ulrike, Sos, Martin L., Liao, Sida, Wu, Catherine J., Polyak, Kornelia, Piha-Paul, Sarina A. and Shapiro, Geoffrey I. (2022). Report of the First International Symposium on NUT Carcinoma. Clin. Cancer Res., 28 (12). S. 2493 - 2506. PHILADELPHIA: AMER ASSOC CANCER RESEARCH. ISSN 1557-3265

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Abstract

NUT carcinoma is a rare, aggressive cancer defined by rearrangements of the NUTM1 gene. No routinely effective treatments of NUT carcinoma exist, despite harboring a targetable oncoprotein, most commonly BRD4-NUT. The vast majority of cases are fatal. Poor awareness of the disease is a major obstacle to progress in the treatment of NUT carcinoma. While the incidence likely exceeds that of Ewing sarcoma, and BRD4-NUT heralded the bromodomain and extra-terminal domain (BET) inhibitor class of selective epigenetic modulators, NUT carcinoma is incorrectly perceived as impossibly rare, and therefore receives comparatively little private or governmental funding or prioritization by pharma. To raise awareness, propagate scientific knowledge, and initiate a consensus on standard and targeted treatment of NUT carcinoma, we held the First International Symposium on NUT Carcinoma on March 3, 2021. This virtual event had more than eighty attendees from the Americas, Europe, Asia, and Australia. Patients with NUT carcinoma and family members were represented and shared perspectives. Broadly, the four areas discussed by experts in the field included (1) the biology of NUT carcinoma; (2) standard approaches to the treatment of NUT carcinoma; (3) results of clinical trials using BET inhibitors; and (4) future directions, including novel BET bromodomain inhibitors, combinatorial approaches, and immunotherapy. It was concluded that standard chemotherapeutic approaches and first-generation BET bromodomain inhibitors, the latter complicated by a narrow therapeutic window, are only modestly effective in a minority of cases. Nonetheless, emerging second-generation targeted inhibitors, novel rational synergistic combinations, and the incorporation of immuno-oncology approaches hold promise to improve the prognosis of this disease.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
French, Christopher A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cheng, Michael L.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hanna, Glenn J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
DuBois, Steven G.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Chau, Nicole G.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hann, Christine L.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Storck, SimoneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Salgia, RaviUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Trucco, MatteoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tseng, JenniferUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stathis, AnastasiosUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Piekarz, RichardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lauer, Ulrich M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Massard, ChristopheUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bennett, KellyUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Coker, ShodeindeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tontsch-Grunt, UlrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sos, Martin L.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Liao, SidaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wu, Catherine J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Polyak, KorneliaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Piha-Paul, Sarina A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Shapiro, Geoffrey I.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-696973
DOI: 10.1158/1078-0432.CCR-22-0591
Journal or Publication Title: Clin. Cancer Res.
Volume: 28
Number: 12
Page Range: S. 2493 - 2506
Date: 2022
Publisher: AMER ASSOC CANCER RESEARCH
Place of Publication: PHILADELPHIA
ISSN: 1557-3265
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
BET BROMODOMAIN INHIBITION; CELL-CYCLE PROGRESSION; MIDLINE CARCINOMA; SYNTHETIC LETHAL; SELECTIVE-INHIBITION; BRD4 INHIBITION; PROTEIN BRD4; P-TEFB; MYC; TRANSCRIPTIONMultiple languages
OncologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/69697

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